6H03 | pdb_00006h03

OPEN CONFORMATION OF THE MEMBRANE ATTACK COMPLEX

External Resource: Annotation

Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations
Membrane Protein Annotation: OPM
Membrane Protein Annotation: mpstruc

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
B [auth C]	SCOP2B Superfamily	LDL receptor-like module	8052139	3000728	SCOP2B (2022-06-29)
B [auth C]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052149	3000814	SCOP2B (2022-06-29)
B [auth C]	SCOP2B Superfamily	MAC/CDC-like	8052046	3002081	SCOP2B (2022-06-29)
B [auth C]	SCOP2B Superfamily	EGF/Laminin-like	8052131	3000412	SCOP2B (2022-06-29)
D [auth E]	SCOP2B Superfamily	Lipocalins	8041276	3001332	SCOP2B (2022-06-29)
E [auth F]	SCOP2B Superfamily	MAC/CDC-like	8052045	3002081	SCOP2B (2022-06-29)
E [auth F]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052147	3000814	SCOP2B (2022-06-29)
E [auth F]	SCOP2B Superfamily	LDL receptor-like module	8052137	3000728	SCOP2B (2022-06-29)
E [auth F]	SCOP2B Superfamily	EGF/Laminin-like	8052128	3000412	SCOP2B (2022-06-29)
F [auth B]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052120	3000814	SCOP2B (2022-06-29)
F [auth B]	SCOP2B Superfamily	LDL receptor-like module	8052133	3000728	SCOP2B (2022-06-29)
F [auth B]	SCOP2B Superfamily	Sushi/CCP/SCR domain	8052110	3000902	SCOP2B (2022-06-29)
F [auth B]	SCOP2B Superfamily	Sushi/CCP/SCR domain	8052111	3000902	SCOP2B (2022-06-29)
F [auth B]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052119	3000814	SCOP2B (2022-06-29)
F [auth B]	SCOP2B Superfamily	MAC/CDC-like	8052040	3002081	SCOP2B (2022-06-29)
G	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
G	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
G	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
G	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
H [auth P]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
H [auth P]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
H [auth P]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
H [auth P]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
I [auth H]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
I [auth H]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
I [auth H]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
I [auth H]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
J [auth I]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
J [auth I]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
J [auth I]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
J [auth I]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
K [auth J]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
K [auth J]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
K [auth J]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
K [auth J]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
L [auth K]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
L [auth K]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
L [auth K]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
L [auth K]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
M [auth L]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
M [auth L]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
M [auth L]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
M [auth L]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
N [auth M]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
N [auth M]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
N [auth M]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
N [auth M]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
O [auth N]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
O [auth N]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
O [auth N]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
O [auth N]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
P [auth O]	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
P [auth O]	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
P [auth O]	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
P [auth O]	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
Q	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
Q	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
Q	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
Q	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
R	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
R	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
R	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
R	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
S	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
S	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
S	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
S	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
T	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
T	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
T	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
T	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
U	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
U	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
U	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
U	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
V	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
V	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
V	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
V	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
W	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
W	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
W	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)
W	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
X	SCOP2B Superfamily	TSP-1 type 1 repeat	8052127	3000814	SCOP2B (2022-06-29)
X	SCOP2B Superfamily	EGF/Laminin-like	8052125	3000412	SCOP2B (2022-06-29)
X	SCOP2B Superfamily	LDL receptor-like module	8052141	3000728	SCOP2B (2022-06-29)
X	SCOP2B Superfamily	MAC/CDC-like	8052039	3002081	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
B [auth C]	MACPF	e6h03C5	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
B [auth C]	hEGF_like	e6h03C1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_like	ECOD (1.6)
B [auth C]	Ldl_recept_a	e6h03C3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
B [auth C]	TSP_1_1	e6h03C4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
B [auth C]	TSP_1	e6h03C2	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1	ECOD (1.6)
C [auth D]	MACPF	e6h03D1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
C [auth D]	hEGF_like	e6h03D7	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_like	ECOD (1.6)
C [auth D]	Sushi	e6h03D6	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: Complement control module/SCR domain	F: Sushi	ECOD (1.6)
C [auth D]	Sushi_1	e6h03D4	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: Complement control module/SCR domain	F: Sushi_1	ECOD (1.6)
C [auth D]	Ldl_recept_a	e6h03D5	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
C [auth D]	TSP_1_1	e6h03D3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
C [auth D]	TSP_1	e6h03D2	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1	ECOD (1.6)
D [auth E]	Lipocalin	e6h03E1	A: beta barrels	X: Lipocalins/Streptavidin	H: Lipocalins (From Topology)	T: Lipocalins	F: Lipocalin	ECOD (1.6)
E [auth F]	MACPF	e6h03F2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
E [auth F]	hEGF_like	e6h03F1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_like	ECOD (1.6)
E [auth F]	Ldl_recept_a	e6h03F3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
E [auth F]	TSP_1_1	e6h03F5	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
F [auth B]	MACPF	e6h03B1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
F [auth B]	hEGF_like	e6h03B8	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_like	ECOD (1.6)
F [auth B]	Sushi	e6h03B3	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: Complement control module/SCR domain	F: Sushi	ECOD (1.6)
F [auth B]	Ldl_recept_a	e6h03B7	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
F [auth B]	TSP_1_1	e6h03B6	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
F [auth B]	TSP_1	e6h03B5	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1	ECOD (1.6)
G	MACPF	e6h03G1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
G	hEGF_4	e6h03G3	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
G	Ldl_recept_a	e6h03G2	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
G	TSP_1_1	e6h03G4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
H [auth P]	MACPF	e6h03P2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
H [auth P]	hEGF_4	e6h03P3	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
H [auth P]	Ldl_recept_a	e6h03P4	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
H [auth P]	TSP_1_1	e6h03P1	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
I [auth H]	MACPF	e6h03H2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
I [auth H]	hEGF_4	e6h03H1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
I [auth H]	Ldl_recept_a	e6h03H3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
I [auth H]	TSP_1_1	e6h03H4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
J [auth I]	MACPF	e6h03I2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
J [auth I]	hEGF_4	e6h03I3	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
J [auth I]	Ldl_recept_a	e6h03I1	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
J [auth I]	TSP_1_1	e6h03I4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
K [auth J]	MACPF	e6h03J4	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
K [auth J]	hEGF_4	e6h03J2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
K [auth J]	Ldl_recept_a	e6h03J1	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
K [auth J]	TSP_1_1	e6h03J3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
L [auth K]	MACPF	e6h03K1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
L [auth K]	hEGF_4	e6h03K4	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
L [auth K]	Ldl_recept_a	e6h03K2	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
L [auth K]	TSP_1_1	e6h03K3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
M [auth L]	MACPF	e6h03L4	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
M [auth L]	hEGF_4	e6h03L1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
M [auth L]	Ldl_recept_a	e6h03L2	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
M [auth L]	TSP_1_1	e6h03L3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
N [auth M]	MACPF	e6h03M1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
N [auth M]	hEGF_4	e6h03M4	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
N [auth M]	Ldl_recept_a	e6h03M3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
N [auth M]	TSP_1_1	e6h03M2	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
O [auth N]	MACPF	e6h03N2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
O [auth N]	hEGF_4	e6h03N1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
O [auth N]	Ldl_recept_a	e6h03N3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
O [auth N]	TSP_1_1	e6h03N4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
P [auth O]	MACPF	e6h03O2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
P [auth O]	hEGF_4	e6h03O3	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
P [auth O]	Ldl_recept_a	e6h03O4	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
P [auth O]	TSP_1_1	e6h03O1	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
Q	MACPF	e6h03Q2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
Q	hEGF_4	e6h03Q1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
Q	Ldl_recept_a	e6h03Q3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
Q	TSP_1_1	e6h03Q4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
R	MACPF	e6h03R3	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
R	hEGF_4	e6h03R2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
R	Ldl_recept_a	e6h03R4	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
R	TSP_1_1	e6h03R1	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
S	MACPF	e6h03S2	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
S	hEGF_4	e6h03S4	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
S	Ldl_recept_a	e6h03S1	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
S	TSP_1_1	e6h03S3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
T	MACPF	e6h03T1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
T	hEGF_4	e6h03T3	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
T	Ldl_recept_a	e6h03T4	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
T	TSP_1_1	e6h03T2	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
U	MACPF	e6h03U4	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
U	hEGF_4	e6h03U1	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
U	Ldl_recept_a	e6h03U2	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
U	TSP_1_1	e6h03U3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
V	MACPF	e6h03V1	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
V	hEGF_4	e6h03V4	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
V	Ldl_recept_a	e6h03V2	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
V	TSP_1_1	e6h03V3	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
W	MACPF	e6h03W4	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
W	hEGF_4	e6h03W2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
W	Ldl_recept_a	e6h03W3	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
W	TSP_1_1	e6h03W1	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)
X	MACPF	e6h03X3	A: beta complex topology	X: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	H: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains (From Topology)	T: Membrane attack complex/perforin (MACPF) and cholesterol-dependent cytolysin (CDC) domains	F: MACPF	ECOD (1.6)
X	hEGF_4	e6h03X2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: hEGF_4	ECOD (1.6)
X	Ldl_recept_a	e6h03X1	A: few secondary structure elements	X: EGF-like	H: LDL receptor-like module (From Topology)	T: LDL receptor-like module	F: Ldl_recept_a	ECOD (1.6)
X	TSP_1_1	e6h03X4	A: few secondary structure elements	X: TSP-1 type 1 repeat (From Topology)	H: TSP-1 type 1 repeat (From Topology)	T: TSP-1 type 1 repeat	F: TSP_1_1	ECOD (1.6)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF17791	Macroglobulin domain MG3 (MG3)	Macroglobulin domain MG3	This entry corresponds to the MG3 domain found in complement components C3, C4 and C5.	Domain
A	PF01835	MG2 domain (MG2)	MG2 domain	This is the MG2 (macroglobulin) domain of alpha-2-macroglobulin in eukaryotes [1]. Alpha-2-macroglobulins (A2Ms) are plasma proteins that trap and inhibit a broad range of proteases and are major components of the eukaryotic innate immune system. How ...	This is the MG2 (macroglobulin) domain of alpha-2-macroglobulin in eukaryotes [1]. Alpha-2-macroglobulins (A2Ms) are plasma proteins that trap and inhibit a broad range of proteases and are major components of the eukaryotic innate immune system. However, A2M-like proteins were identified in pathogenically invasive bacteria and species that colonize higher eukaryotes. This domain is found in eukaryotic and bacterial proteins. In human A2Ms, this domain is termed macroglobulin-like (MG) domain 2 and in Salmonella enterica ser A2Ms, this is domain 4 [2] [3].	Domain
A	PF00207	Alpha-2-macroglobulin family (A2M)	Alpha-2-macroglobulin family	-	Family
A	PF07678	A-macroglobulin TED domain (TED_complement)	A-macroglobulin TED domain	-	Repeat
A	PF21309	Complement component 5, CUB domain (C5_CUB)	Complement component 5, CUB domain	Complement component 5 (C5) is a component of the lytic complex consisting of factors C5-C9 which attack bacterial cell membranes. This entry represents the CUB domain [1-5].	Domain
A	PF17789	Macroglobulin domain MG4 (MG4)	Macroglobulin domain MG4	This domain is MG4 found in complement C3 and C5 proteins.	Domain
A	PF07703	Alpha-2-macroglobulin bait region domain (A2M_BRD)	Alpha-2-macroglobulin bait region domain	Alpha-2-macroglobulins (A2Ms) are plasma proteins that trap and inhibit a broad range of proteases and are major components of the eukaryotic innate immune system. However, A2M-like proteins were identified in pathogenically invasive bacteria and spe ...	Alpha-2-macroglobulins (A2Ms) are plasma proteins that trap and inhibit a broad range of proteases and are major components of the eukaryotic innate immune system. However, A2M-like proteins were identified in pathogenically invasive bacteria and species that colonize higher eukaryotes. This domain is found in eukaryotic and bacterial proteins. In human A2Ms, this domain encompasses macroglobulin-like domain MG5 and 6 including bait region. In Salmonella enterica ser A2Ms, this domain encompasses MG7 and MG8 including the bait region [1] [2]. The Bait region is cleaved by proteases, followed by a large conformational change that blocks the target protease within a cage-like complex. This model of protease entrapment is recognised as the Venus flytrap mechanism [1].	Domain
B [auth C]	PF00090	Thrombospondin type 1 domain (TSP_1)	Thrombospondin type 1 domain		Domain
B [auth C]	PF01823	MAC/Perforin domain (MACPF)	MAC/Perforin domain	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Fr ...	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-7 complex. The C5b-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-8 complex. C5b-8 subsequently binds to C9 and acts as a catalyst in the polymerisation of C9. Active MAC has a subunit composition of C5b-C6-C7-C8-C9{n}. Perforin is a protein found in cytolytic T-cell and killer cells. In the presence of calcium, perforin polymerises into transmembrane tubules and is capable of lysing, non-specifically, a variety of target cells. There are a number of regions of similarity in the sequences of complement components C6, C7, C8-alpha, C8-beta, C9 and perforin. The X-ray crystal structure of a MACPF domain reveals that it shares a common fold with bacterial cholesterol dependent cytolysins (Pfam:PF01289) such as perfringolysin O. Three key pieces of evidence suggests that MACPF domains and CDCs are homologous: Functional similarity (pore formation), conservation of three glycine residues at a hinge in both families and conservation of a complex core fold [1].	Domain
B [auth C]	PF21195	Complement components C8A/B/C6, EGF-like domain (EGF_C8A_B_C6)	Complement components C8A/B/C6, EGF-like domain	This domain is found in the alpha and beta chains of Complement component C8 (C8A/B), in Complement components C6 (C6) from humans, constituents of the membrane attack complex (MAC) that play a key role in the innate and adaptive immune response by f ...	This domain is found in the alpha and beta chains of Complement component C8 (C8A/B), in Complement components C6 (C6) from humans, constituents of the membrane attack complex (MAC) that play a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. This entry represents the rigid, disulfide-rich epidermal growth factor (EGF) domain, which is included in the regulatory segment of these proteins [1,2]. It is found C-terminal to the membrane attack complex perforin domain Pfam:PF01823 and N-terminal to the thrombospondin type 1 domain Pfam:PF00090.	Domain
B [auth C]	PF00057	Low-density lipoprotein receptor domain class A (Ldl_recept_a)	Low-density lipoprotein receptor domain class A	-	Repeat
C [auth D]	PF00090	Thrombospondin type 1 domain (TSP_1)	Thrombospondin type 1 domain		Domain
C [auth D]	PF01823	MAC/Perforin domain (MACPF)	MAC/Perforin domain	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Fr ...	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-7 complex. The C5b-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-8 complex. C5b-8 subsequently binds to C9 and acts as a catalyst in the polymerisation of C9. Active MAC has a subunit composition of C5b-C6-C7-C8-C9{n}. Perforin is a protein found in cytolytic T-cell and killer cells. In the presence of calcium, perforin polymerises into transmembrane tubules and is capable of lysing, non-specifically, a variety of target cells. There are a number of regions of similarity in the sequences of complement components C6, C7, C8-alpha, C8-beta, C9 and perforin. The X-ray crystal structure of a MACPF domain reveals that it shares a common fold with bacterial cholesterol dependent cytolysins (Pfam:PF01289) such as perfringolysin O. Three key pieces of evidence suggests that MACPF domains and CDCs are homologous: Functional similarity (pore formation), conservation of three glycine residues at a hinge in both families and conservation of a complex core fold [1].	Domain
C [auth D]	PF00084	Sushi repeat (SCR repeat) (Sushi)	Sushi repeat (SCR repeat)		Domain
C [auth D]	PF00057	Low-density lipoprotein receptor domain class A (Ldl_recept_a)	Low-density lipoprotein receptor domain class A	-	Repeat
D [auth E]	PF00061	Lipocalin / cytosolic fatty-acid binding protein family (Lipocalin)	Lipocalin / cytosolic fatty-acid binding protein family	Lipocalins are transporters for small hydrophobic molecules, such as lipids, steroid hormones, bilins, and retinoids. The family also encompasses the enzyme prostaglandin D synthase (EC:5.3.99.2). Alignment subsumes both the lipocalin and fatty acid ...	Lipocalins are transporters for small hydrophobic molecules, such as lipids, steroid hormones, bilins, and retinoids. The family also encompasses the enzyme prostaglandin D synthase (EC:5.3.99.2). Alignment subsumes both the lipocalin and fatty acid binding protein signatures from PROSITE. This is supported on structural and functional grounds. The structure is an eight-stranded beta barrel.	Domain
E [auth F]	PF00090	Thrombospondin type 1 domain (TSP_1)	Thrombospondin type 1 domain		Domain
E [auth F]	PF01823	MAC/Perforin domain (MACPF)	MAC/Perforin domain	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Fr ...	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-7 complex. The C5b-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-8 complex. C5b-8 subsequently binds to C9 and acts as a catalyst in the polymerisation of C9. Active MAC has a subunit composition of C5b-C6-C7-C8-C9{n}. Perforin is a protein found in cytolytic T-cell and killer cells. In the presence of calcium, perforin polymerises into transmembrane tubules and is capable of lysing, non-specifically, a variety of target cells. There are a number of regions of similarity in the sequences of complement components C6, C7, C8-alpha, C8-beta, C9 and perforin. The X-ray crystal structure of a MACPF domain reveals that it shares a common fold with bacterial cholesterol dependent cytolysins (Pfam:PF01289) such as perfringolysin O. Three key pieces of evidence suggests that MACPF domains and CDCs are homologous: Functional similarity (pore formation), conservation of three glycine residues at a hinge in both families and conservation of a complex core fold [1].	Domain
E [auth F]	PF21195	Complement components C8A/B/C6, EGF-like domain (EGF_C8A_B_C6)	Complement components C8A/B/C6, EGF-like domain	This domain is found in the alpha and beta chains of Complement component C8 (C8A/B), in Complement components C6 (C6) from humans, constituents of the membrane attack complex (MAC) that play a key role in the innate and adaptive immune response by f ...	This domain is found in the alpha and beta chains of Complement component C8 (C8A/B), in Complement components C6 (C6) from humans, constituents of the membrane attack complex (MAC) that play a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. This entry represents the rigid, disulfide-rich epidermal growth factor (EGF) domain, which is included in the regulatory segment of these proteins [1,2]. It is found C-terminal to the membrane attack complex perforin domain Pfam:PF01823 and N-terminal to the thrombospondin type 1 domain Pfam:PF00090.	Domain
F [auth B]	PF00090	Thrombospondin type 1 domain (TSP_1)	Thrombospondin type 1 domain		Domain
F [auth B]	PF01823	MAC/Perforin domain (MACPF)	MAC/Perforin domain	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Fr ...	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-7 complex. The C5b-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-8 complex. C5b-8 subsequently binds to C9 and acts as a catalyst in the polymerisation of C9. Active MAC has a subunit composition of C5b-C6-C7-C8-C9{n}. Perforin is a protein found in cytolytic T-cell and killer cells. In the presence of calcium, perforin polymerises into transmembrane tubules and is capable of lysing, non-specifically, a variety of target cells. There are a number of regions of similarity in the sequences of complement components C6, C7, C8-alpha, C8-beta, C9 and perforin. The X-ray crystal structure of a MACPF domain reveals that it shares a common fold with bacterial cholesterol dependent cytolysins (Pfam:PF01289) such as perfringolysin O. Three key pieces of evidence suggests that MACPF domains and CDCs are homologous: Functional similarity (pore formation), conservation of three glycine residues at a hinge in both families and conservation of a complex core fold [1].	Domain
F [auth B]	PF21195	Complement components C8A/B/C6, EGF-like domain (EGF_C8A_B_C6)	Complement components C8A/B/C6, EGF-like domain	This domain is found in the alpha and beta chains of Complement component C8 (C8A/B), in Complement components C6 (C6) from humans, constituents of the membrane attack complex (MAC) that play a key role in the innate and adaptive immune response by f ...	This domain is found in the alpha and beta chains of Complement component C8 (C8A/B), in Complement components C6 (C6) from humans, constituents of the membrane attack complex (MAC) that play a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells. This entry represents the rigid, disulfide-rich epidermal growth factor (EGF) domain, which is included in the regulatory segment of these proteins [1,2]. It is found C-terminal to the membrane attack complex perforin domain Pfam:PF01823 and N-terminal to the thrombospondin type 1 domain Pfam:PF00090.	Domain
F [auth B]	PF00084	Sushi repeat (SCR repeat) (Sushi)	Sushi repeat (SCR repeat)		Domain
F [auth B]	PF00057	Low-density lipoprotein receptor domain class A (Ldl_recept_a)	Low-density lipoprotein receptor domain class A	-	Repeat
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	PF00090	Thrombospondin type 1 domain (TSP_1)	Thrombospondin type 1 domain		Domain
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	PF01823	MAC/Perforin domain (MACPF)	MAC/Perforin domain	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Fr ...	The membrane-attack complex (MAC) of the complement system forms transmembrane channels. These channels disrupt the phospholipid bilayer of target cells, leading to cell lysis and death. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-7 complex. The C5b-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-8 complex. C5b-8 subsequently binds to C9 and acts as a catalyst in the polymerisation of C9. Active MAC has a subunit composition of C5b-C6-C7-C8-C9{n}. Perforin is a protein found in cytolytic T-cell and killer cells. In the presence of calcium, perforin polymerises into transmembrane tubules and is capable of lysing, non-specifically, a variety of target cells. There are a number of regions of similarity in the sequences of complement components C6, C7, C8-alpha, C8-beta, C9 and perforin. The X-ray crystal structure of a MACPF domain reveals that it shares a common fold with bacterial cholesterol dependent cytolysins (Pfam:PF01289) such as perfringolysin O. Three key pieces of evidence suggests that MACPF domains and CDCs are homologous: Functional similarity (pore formation), conservation of three glycine residues at a hinge in both families and conservation of a complex core fold [1].	Domain
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	PF00057	Low-density lipoprotein receptor domain class A (Ldl_recept_a)	Low-density lipoprotein receptor domain class A	-	Repeat

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	Complement C5,Complement C5	protein binding binding signaling receptor binding molecular transducer activity molecular function activator activity signaling receptor activity G protein-coupled receptor binding signaling receptor activator activity cytokine receptor binding chemokine activity molecular function regulator activity cytokine activity signaling receptor regulator activity chemokine receptor binding protein binding binding signaling receptor binding molecular transducer activity molecular function activator activity signaling receptor activity G protein-coupled receptor binding signaling receptor activator activity cytokine receptor binding chemokine activity molecular function regulator activity cytokine activity signaling receptor regulator activity chemokine receptor binding receptor ligand activity enzyme regulator activity peptidase activity peptidase regulator activity catalytic activity, acting on a protein hydrolase activity enzyme inhibitor activity peptidase inhibitor activity endopeptidase regulator activity endopeptidase inhibitor activity molecular function inhibitor activity catalytic activity endopeptidase activity	adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response response to stimulus positive regulation of biological process positive regulation of response to stimulus complement activation humoral immune response mediated by circulating immunoglobulin positive regulation of immune response leukocyte mediated immunity regulation of immune system process positive regulation of immune system process complement activation, classical pathway regulation of biological process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response response to stimulus positive regulation of biological process positive regulation of response to stimulus complement activation humoral immune response mediated by circulating immunoglobulin positive regulation of immune response leukocyte mediated immunity regulation of immune system process positive regulation of immune system process complement activation, classical pathway regulation of biological process immune response biological regulation immunoglobulin mediated immune response adaptive immune response immune effector process regulation of response to stimulus immune system process B cell mediated immunity regulation of immune response activation of immune response cellular response to stimulus negative regulation of mononuclear cell migration negative regulation of locomotion regulation of cell motility regulation of cellular process negative regulation of biological process negative regulation of chemotaxis macrophage migration taxis negative regulation of macrophage chemotaxis cell migration regulation of mononuclear cell migration negative regulation of macrophage migration negative regulation of cell migration chemotaxis regulation of leukocyte chemotaxis regulation of leukocyte migration response to external stimulus leukocyte migration negative regulation of leukocyte migration regulation of locomotion response to chemical negative regulation of response to external stimulus negative regulation of cell motility negative regulation of leukocyte chemotaxis regulation of response to external stimulus macrophage chemotaxis regulation of macrophage migration negative regulation of cellular process regulation of cell migration myeloid leukocyte migration cell motility locomotion cell chemotaxis regulation of macrophage chemotaxis negative regulation of response to stimulus regulation of chemotaxis cellular response to chemical stimulus negative regulation of immune system process mononuclear cell migration cellular process leukocyte chemotaxis multicellular organismal process positive regulation of macromolecule biosynthetic process positive regulation of metabolic process positive regulation of biosynthetic process regulation of cytokine production cytokine production regulation of multicellular organismal process positive regulation of macromolecule metabolic process positive regulation of cellular process regulation of vascular endothelial growth factor production regulation of biosynthetic process regulation of macromolecule metabolic process gene expression macromolecule biosynthetic process regulation of gene expression vascular endothelial growth factor production biosynthetic process positive regulation of gene expression positive regulation of multicellular organismal process positive regulation of vascular endothelial growth factor production regulation of macromolecule biosynthetic process metabolic process macromolecule metabolic process positive regulation of cytokine production regulation of metabolic process regulation of chemokine production chemokine production positive regulation of chemokine production cell surface receptor signaling pathway signaling signal transduction cell communication biological process involved in interspecies interaction between organisms disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing defense response response to stress inflammatory response G protein-coupled receptor signaling pathway complement activation, alternative pathway response to biotic stimulus innate immune response response to external biotic stimulus response to other organism defense response to symbiont defense response to other organism	extracellular exosome membrane-bounded organelle extracellular vesicle cellular anatomical structure extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane extracellular exosome membrane-bounded organelle extracellular vesicle cellular anatomical structure extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane plasma membrane membrane protein complex pore complex membrane attack complex
B [auth C]	Complement component C8 beta chain	protein-containing complex binding binding	regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response complement activation humoral immune response mediated by circulating immunoglobulin leukocyte mediated immunity complement activation, classical pathway immunoglobulin mediated immune response adaptive immune response immune effector process B cell mediated immunity activation of immune response biological process involved in interspecies interaction between organisms cellular process disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing complement activation, alternative pathway response to biotic stimulus response to stress innate immune response response to external biotic stimulus response to other organism defense response defense response to symbiont response to external stimulus defense response to other organism	cellular anatomical structure membrane extracellular region extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex cellular anatomical structure membrane extracellular region extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex plasma membrane membrane protein complex pore complex membrane attack complex
C [auth D]	Complement component C7	-	regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response complement activation humoral immune response mediated by circulating immunoglobulin leukocyte mediated immunity complement activation, classical pathway immunoglobulin mediated immune response adaptive immune response immune effector process B cell mediated immunity activation of immune response biological process involved in interspecies interaction between organisms cellular process disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing complement activation, alternative pathway response to biotic stimulus response to stress innate immune response response to external biotic stimulus response to other organism defense response defense response to symbiont response to external stimulus defense response to other organism	cellular anatomical structure extracellular region extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane cellular anatomical structure extracellular region extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane plasma membrane membrane protein complex pore complex membrane attack complex
D [auth E]	Complement component C8 gamma chain	protein-containing complex binding binding vitamin binding alcohol binding isoprenoid binding retinoid binding lipid binding retinol binding small molecule binding protein binding complement binding	regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response complement activation humoral immune response mediated by circulating immunoglobulin leukocyte mediated immunity complement activation, classical pathway immunoglobulin mediated immune response adaptive immune response immune effector process B cell mediated immunity activation of immune response biological process involved in interspecies interaction between organisms cellular process disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing complement activation, alternative pathway response to biotic stimulus response to stress innate immune response response to external biotic stimulus response to other organism defense response defense response to symbiont response to external stimulus defense response to other organism	cellular anatomical structure extracellular region extracellular space blood microparticle extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex cellular anatomical structure extracellular region extracellular space blood microparticle extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane plasma membrane membrane protein complex pore complex membrane attack complex
E [auth F]	Complement component C8 alpha chain	protein binding binding complement binding protein-containing complex binding	regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response complement activation humoral immune response mediated by circulating immunoglobulin leukocyte mediated immunity complement activation, classical pathway immunoglobulin mediated immune response adaptive immune response immune effector process B cell mediated immunity activation of immune response biological process involved in interspecies interaction between organisms cellular process disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing complement activation, alternative pathway response to biotic stimulus response to stress innate immune response response to external biotic stimulus response to other organism defense response defense response to symbiont response to external stimulus defense response to other organism	cellular anatomical structure membrane extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex cellular anatomical structure membrane extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex plasma membrane membrane protein complex pore complex membrane attack complex blood microparticle
F [auth B]	Complement component C6	-	regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response complement activation humoral immune response mediated by circulating immunoglobulin leukocyte mediated immunity complement activation, classical pathway immunoglobulin mediated immune response adaptive immune response immune effector process B cell mediated immunity activation of immune response biological process involved in interspecies interaction between organisms response to other organism defense response defense response to symbiont response to external stimulus response to biotic stimulus defense response to other organism response to stress innate immune response response to external biotic stimulus developmental process multicellular organism development multicellular organismal process embryo development ending in birth or egg hatching chordate embryonic development anatomical structure development embryo development in utero embryonic development cellular process disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing	extracellular exosome membrane-bounded organelle extracellular vesicle cellular anatomical structure extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane extracellular exosome membrane-bounded organelle extracellular vesicle cellular anatomical structure extracellular organelle extracellular region organelle extracellular space vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane plasma membrane membrane protein complex pore complex membrane attack complex
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	Complement component C9	-	regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity regulation of biological process response to stimulus immune response biological regulation positive regulation of biological process positive regulation of response to stimulus positive regulation of immune response regulation of response to stimulus immune system process regulation of immune response regulation of immune system process positive regulation of immune system process adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains lymphocyte mediated immunity humoral immune response complement activation humoral immune response mediated by circulating immunoglobulin leukocyte mediated immunity complement activation, classical pathway immunoglobulin mediated immune response adaptive immune response immune effector process B cell mediated immunity activation of immune response biological process involved in interspecies interaction between organisms cellular process disruption of cell in another organism disruption of anatomical structure in another organism killing of cells of another organism cell killing complement activation, alternative pathway response to biotic stimulus response to stress innate immune response response to external biotic stimulus response to other organism defense response defense response to symbiont response to external stimulus defense response to other organism cellular component organization cellular component biogenesis cellular component assembly protein-containing complex organization protein complex oligomerization protein-containing complex assembly protein homooligomerization cellular component organization or biogenesis	cellular anatomical structure extracellular region extracellular space blood microparticle extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex cellular anatomical structure extracellular region extracellular space blood microparticle extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle organelle vesicle extracellular membrane-bounded organelle cell periphery protein-containing complex plasma membrane protein complex membrane plasma membrane membrane protein complex pore complex membrane attack complex other organism cell membrane other organism part

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR009048	Alpha-macroglobulin, receptor-binding	Domain
A	IPR041555	Macroglobulin domain MG3	Domain
A	IPR001599	Alpha-2-macroglobulin	Domain
A	IPR048843	Complement component 5, CUB domain	Domain
A	IPR018081	Anaphylatoxin, complement system	Homologous Superfamily
A	IPR018933	Netrin module, non-TIMP type	Domain
A	IPR001840	Anaphylatoxin, complement system domain	Domain
A	IPR041425	Complement C3/4/5, macroglobulin domain MG1	Domain
A	IPR011625	Alpha-2-macroglobulin, bait region domain	Domain
A	IPR001134	Netrin domain	Domain
A	IPR008993	Tissue inhibitor of metalloproteinases-like, OB-fold	Homologous Superfamily
A	IPR011626	Alpha-macroglobulin-like, TED domain	Domain
A	IPR000020	Anaphylatoxin/fibulin	Domain
A	IPR036595	Alpha-macroglobulin, receptor-binding domain superfamily	Homologous Superfamily
A	IPR002890	Macroglobulin domain	Domain
A	IPR013783	Immunoglobulin-like fold	Homologous Superfamily
A	IPR050473	Alpha-2-macroglobulin/Complement system	Family
A	IPR008930	Terpenoid cyclases/protein prenyltransferase alpha-alpha toroid	Homologous Superfamily
A	IPR040839	Macroglobulin domain MG4	Domain
B [auth C]	IPR000884	Thrombospondin type-1 (TSP1) repeat	Repeat
B [auth C]	IPR020864	Membrane attack complex component/perforin (MACPF) domain	Domain
B [auth C]	IPR002172	Low-density lipoprotein (LDL) receptor class A repeat	Repeat
B [auth C]	IPR036383	Thrombospondin type-1 (TSP1) repeat superfamily	Homologous Superfamily
B [auth C]	IPR001862	Membrane attack complex component/perforin/complement C9	Family
B [auth C]	IPR048831	Complement components C8A/B/C6, EGF-like domain	Domain
B [auth C]	IPR023415	Low-density lipoprotein (LDL) receptor class A, conserved site	Conserved Site
B [auth C]	IPR036055	LDL receptor-like superfamily	Homologous Superfamily
B [auth C]	IPR020863	Membrane attack complex component/perforin domain, conserved site	Conserved Site
C [auth D]	IPR000884	Thrombospondin type-1 (TSP1) repeat	Repeat
C [auth D]	IPR020864	Membrane attack complex component/perforin (MACPF) domain	Domain
C [auth D]	IPR002172	Low-density lipoprotein (LDL) receptor class A repeat	Repeat
C [auth D]	IPR040729	Kazal-type serine protease inhibitor domain	Domain
C [auth D]	IPR036383	Thrombospondin type-1 (TSP1) repeat superfamily	Homologous Superfamily
C [auth D]	IPR048827	Complement component C7, FIM2 N-terminal domain	Domain
C [auth D]	IPR001862	Membrane attack complex component/perforin/complement C9	Family
C [auth D]	IPR023415	Low-density lipoprotein (LDL) receptor class A, conserved site	Conserved Site
C [auth D]	IPR036055	LDL receptor-like superfamily	Homologous Superfamily
C [auth D]	IPR020863	Membrane attack complex component/perforin domain, conserved site	Conserved Site
C [auth D]	IPR000436	Sushi/SCR/CCP domain	Domain
C [auth D]	IPR048825	Complement component C7, Kazal domain	Domain
C [auth D]	IPR003884	Factor I / membrane attack complex	Domain
C [auth D]	IPR035976	Sushi/SCR/CCP superfamily	Homologous Superfamily
D [auth E]	IPR002968	Alpha-1-microglobulin	Family
D [auth E]	IPR012674	Calycin	Homologous Superfamily
D [auth E]	IPR000566	Lipocalin/cytosolic fatty-acid binding domain	Domain
D [auth E]	IPR043245	Complement component C8 gamma chain	Family
D [auth E]	IPR022272	Lipocalin family conserved site	Conserved Site
E [auth F]	IPR002172	Low-density lipoprotein (LDL) receptor class A repeat	Repeat
E [auth F]	IPR001862	Membrane attack complex component/perforin/complement C9	Family
E [auth F]	IPR036055	LDL receptor-like superfamily	Homologous Superfamily
E [auth F]	IPR020863	Membrane attack complex component/perforin domain, conserved site	Conserved Site
E [auth F]	IPR000884	Thrombospondin type-1 (TSP1) repeat	Repeat
E [auth F]	IPR020864	Membrane attack complex component/perforin (MACPF) domain	Domain
E [auth F]	IPR036383	Thrombospondin type-1 (TSP1) repeat superfamily	Homologous Superfamily
E [auth F]	IPR048831	Complement components C8A/B/C6, EGF-like domain	Domain
E [auth F]	IPR009030	Growth factor receptor cysteine-rich domain superfamily	Homologous Superfamily
E [auth F]	IPR023415	Low-density lipoprotein (LDL) receptor class A, conserved site	Conserved Site
F [auth B]	IPR002172	Low-density lipoprotein (LDL) receptor class A repeat	Repeat
F [auth B]	IPR001862	Membrane attack complex component/perforin/complement C9	Family
F [auth B]	IPR036055	LDL receptor-like superfamily	Homologous Superfamily
F [auth B]	IPR020863	Membrane attack complex component/perforin domain, conserved site	Conserved Site
F [auth B]	IPR048828	Complement component C6, KAZAL domain	Domain
F [auth B]	IPR000436	Sushi/SCR/CCP domain	Domain
F [auth B]	IPR002350	Kazal domain	Domain
F [auth B]	IPR003884	Factor I / membrane attack complex	Domain
F [auth B]	IPR035976	Sushi/SCR/CCP superfamily	Homologous Superfamily
F [auth B]	IPR000884	Thrombospondin type-1 (TSP1) repeat	Repeat
F [auth B]	IPR020864	Membrane attack complex component/perforin (MACPF) domain	Domain
F [auth B]	IPR036383	Thrombospondin type-1 (TSP1) repeat superfamily	Homologous Superfamily
F [auth B]	IPR048831	Complement components C8A/B/C6, EGF-like domain	Domain
F [auth B]	IPR023415	Low-density lipoprotein (LDL) receptor class A, conserved site	Conserved Site
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR000884	Thrombospondin type-1 (TSP1) repeat	Repeat
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR020864	Membrane attack complex component/perforin (MACPF) domain	Domain
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR002172	Low-density lipoprotein (LDL) receptor class A repeat	Repeat
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR036383	Thrombospondin type-1 (TSP1) repeat superfamily	Homologous Superfamily
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR001862	Membrane attack complex component/perforin/complement C9	Family
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR009030	Growth factor receptor cysteine-rich domain superfamily	Homologous Superfamily
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR023415	Low-density lipoprotein (LDL) receptor class A, conserved site	Conserved Site
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR036055	LDL receptor-like superfamily	Homologous Superfamily
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	IPR020863	Membrane attack complex component/perforin domain, conserved site	Conserved Site

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P01031	complement component 5 deficiency rheumatoid arthritis peritonitis primary immunodeficiency disease pancreatic intraductal papillary-mucinous neoplasm psoriasis Secondary Peritonitis acquired paroxysmal nocturnal hemoglobinuria lung cancer malignant pancreatic neoplasm non-small cell lung carcinoma pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia tuberculosis allergic rhinitis complement component 5 deficiency rheumatoid arthritis peritonitis primary immunodeficiency disease pancreatic intraductal papillary-mucinous neoplasm psoriasis Secondary Peritonitis acquired paroxysmal nocturnal hemoglobinuria lung cancer malignant pancreatic neoplasm non-small cell lung carcinoma pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia tuberculosis allergic rhinitis atypical hemolytic-uremic syndrome malignant ependymoma medulloblastoma osteosarcoma pancreatic intraductal papillary-mucinous carcinoma primitive neuroectodermal tumor seborrheic infantile dermatitis atypical teratoid rhabdoid tumor glioblastoma malignant mesothelioma neuromyelitis optica ovarian cancer
B [auth C]	Pharos: P07358	type II complement component 8 deficiency juvenile idiopathic arthritis primary immunodeficiency disease Neisseriaceae Infections lung carcinoma non-small cell lung carcinoma pancreatic ductal adenocarcinoma attention deficit hyperactivity disorder, inattentive type lung adenocarcinoma meningitis ovarian cancer infectious meningitis lung cancer
C [auth D]	Pharos: P10643	complement component 7 deficiency Liver Cirrhosis, Experimental pancreatic intraductal papillary-mucinous neoplasm pituitary cancer ulcerative colitis adrenal cortex adenoma breast carcinoma invasive ductal breast carcinoma lung cancer malignant pancreatic neoplasm non-small cell lung carcinoma pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia psoriasis adrenal cortex carcinoma complement component 7 deficiency Liver Cirrhosis, Experimental pancreatic intraductal papillary-mucinous neoplasm pituitary cancer ulcerative colitis adrenal cortex adenoma breast carcinoma invasive ductal breast carcinoma lung cancer malignant pancreatic neoplasm non-small cell lung carcinoma pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia psoriasis adrenal cortex carcinoma lung carcinoma MALT lymphoma osteosarcoma pancreatic intraductal papillary-mucinous carcinoma pilocytic astrocytoma Sjogren syndrome atopic eczema diabetes mellitus lung adenocarcinoma malignant colon neoplasm medulloblastoma ovarian cancer
D [auth E]	Pharos: P07360	Liver Cirrhosis, Experimental pancreatic ductal adenocarcinoma
E [auth F]	Pharos: P07357	type I complement component 8 deficiency Neisseriaceae Infections primary immunodeficiency disease meningitis pancreatic ductal adenocarcinoma autism spectrum disorder infectious meningitis
F [auth B]	Pharos: P13671	complement component 6 deficiency calcinosis heart valve disease lung carcinoma Microcalcification pancreatic intraductal papillary-mucinous neoplasm invasive ductal breast carcinoma malignant ependymoma pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia ductal breast carcinoma in situ lung adenocarcinoma malignant pancreatic neoplasm pancreatic intraductal papillary-mucinous carcinoma psoriasis
G, H [auth P], I [auth H], J [auth I], K [auth J], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	Pharos: P02748	age-related macular degeneration complement component 9 deficiency meningococcal meningitis dermatomyositis age related macular degeneration 15 opioid abuse primary immunodeficiency disease facial dermatosis Erythema malignant mesothelioma opiate dependence pancreatic ductal adenocarcinoma retinal degeneration exocrine pancreatic carcinoma Finnish type amyloidosis age-related macular degeneration complement component 9 deficiency meningococcal meningitis dermatomyositis age related macular degeneration 15 opioid abuse primary immunodeficiency disease facial dermatosis Erythema malignant mesothelioma opiate dependence pancreatic ductal adenocarcinoma retinal degeneration exocrine pancreatic carcinoma Finnish type amyloidosis wet macular degeneration chronic kidney disease hepatocellular carcinoma macular degeneration psoriasis juvenile dermatomyositis

Membrane Protein Annotation: OPM OPM Database Homepage

Chains	External Link	Type	Class	Superfamily	Family
A, B [auth C], C [auth D], D [auth E], E [auth F] A, B [auth C], C [auth D], D [auth E], E [auth F], F [auth B], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	OPM	Monotopic/peripheral	All beta monotopic/peripheral	Membrane attack complex superfamily	Membrane attack complex(MAC)/perforin

Membrane Protein Annotation: mpstruc mpstruc Database Homepage

Chains	Resource Link	Group	Subgroup	Protein
A, B [auth C], C [auth D], D [auth E], E [auth F] A, B [auth C], C [auth D], D [auth E], E [auth F], F [auth B], G, H [auth P], I [auth H], J [auth I], K [auth J], L [auth K], M [auth L], N [auth M], O [auth N], P [auth O], Q, R, S, T, U, V, W, X	mpstruc	TRANSMEMBRANE PROTEINS: BETA-BARREL	Adventitious Membrane Proteins: Beta-sheet Pore-forming Toxins/Attack Complexes	Membrane attack complex (MAC) in open conformation