4KI5 | pdb_00004ki5

Cystal structure of human factor VIII C2 domain in a ternary complex with murine inhbitory antibodies 3E6 and G99

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
IMGT Antibody Annotation
SAbDab Antibody Annotation
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A [auth C]	d4ki5c_	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	V set domains (antibody variable domain-like)	automated matches	(Mus musculus ) [TaxId: 10090 ],	SCOPe (2.08)
B [auth D]	d4ki5d1	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	automated matches	automated matches	(Mus musculus ) [TaxId: 10090 ],	SCOPe (2.08)
C [auth E]	d4ki5e1	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	automated matches	automated matches	(Mus musculus ) [TaxId: 10090 ],	SCOPe (2.08)
C [auth E]	d4ki5e2	Artifacts	Tags	Tags	Tags	N-terminal Tags	(Mus musculus ) [TaxId: 10090 ],	SCOPe (2.08)
E [auth M]	d4ki5m_	All beta proteins	Galactose-binding domain-like	Galactose-binding domain-like	Discoidin domain (FA58C, coagulation factor 5/8 C-terminal domain)	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
D [auth F]	d4ki5f2	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	C1 set domains (antibody constant domain-like)	automated matches	(Mus musculus ) [TaxId: 10090 ],	SCOPe (2.08)
D [auth F]	d4ki5f1	All beta proteins	Immunoglobulin-like beta-sandwich	Immunoglobulin	automated matches	automated matches	(Mus musculus ) [TaxId: 10090 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
E [auth M]	SCOP2B Superfamily	galactose-binding domain-like	8041137	3000087	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)
A [auth C]	V-set_8	e4ki5C2	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: V-set_8	ECOD (1.6)
A [auth C]	C1-set_1	e4ki5C1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: C1-set_1	ECOD (1.6)
B [auth D]	C1-set_4	e4ki5D1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: C1-set_4	ECOD (1.6)
B [auth D]	V-set	e4ki5D2	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: V-set	ECOD (1.6)
C [auth E]	V-set_8	e4ki5E2	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: V-set_8	ECOD (1.6)
C [auth E]	C1-set_1	e4ki5E1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: C1-set_1	ECOD (1.6)
E [auth M]	F5_F8_type_C	e4ki5M1	A: beta sandwiches	X: jelly-roll	H: Concanavalin A-like	T: Galactose-binding domain-like	F: F5_F8_type_C	ECOD (1.6)
D [auth F]	C1-set_4	e4ki5F1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: C1-set_4	ECOD (1.6)
D [auth F]	V-set	e4ki5F2	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: V-set	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A [auth C]	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)
B [auth D]	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)
C [auth E]	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)
E [auth M]	2.60.120.260	Mainly Beta	Sandwich	Jelly Rolls	Galactose-binding domain-like	CATH (4.3.0)
D [auth F]	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)

IMGT Antibody Annotation IMGT Database Homepage

Chain	Protein Name	Description	Organism Name	Gene Allele Name(s)	Domain Name(s)	Receptor Type	Receptor Description	Provenance Source
A [auth C]	3E6 Fab	VH-CH1	Mus musculus (house mouse)	IGHV9-2-101, IGHJ201, IGHG2A01, IGHG2A02	V-DOMAIN VH, C-DOMAIN CH1	IG	FAB-GAMMA-2A_KAPPA	IMGT (202510-0)
B [auth D]	3E6 Fab	L-KAPPA	Mus musculus (house mouse)	IGKV4-5501, IGKJ101, IGKJ201, IGKJ501, IGKC01, IGKC02	V-DOMAIN V-KAPPA, C-DOMAIN C-KAPPA	IG	FAB-GAMMA-2A_KAPPA	IMGT (202510-0)
C [auth E]	G99 Fab	VH-CH1	Mus musculus (house mouse)	IGHV1-901, IGHJ101, IGHJ102, IGHG2A01, IGHG2A*02	V-DOMAIN VH, C-DOMAIN CH1	IG	FAB-GAMMA-2A_KAPPA	IMGT (202510-0)
D [auth F]	G99 Fab	L-KAPPA	Mus musculus (house mouse)	IGKV9-12401, IGKJ201, IGKC01, IGKC02	V-DOMAIN V-KAPPA, C-DOMAIN C-KAPPA	IG	FAB-GAMMA-2A_KAPPA	IMGT (202510-0)

SAbDab Antibody Annotation SAbDab Database Homepage

Chain	Chain Class	Chain Subclass	Chain Type	Antigen Name(s)	Provenance Source
A [auth C]	Heavy Chain	IGHV9	-	coagulation factor viii	SAbDab (2025-03-21)
B [auth D]	Light Chain	IGKV4	Kappa	coagulation factor viii	SAbDab (2025-03-21)
C [auth E]	Heavy Chain	IGHV1	-	coagulation factor viii	SAbDab (2025-03-21)
D [auth F]	Light Chain	IGKV9	Kappa	coagulation factor viii	SAbDab (2025-03-21)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
E [auth M]	PF00754	F5/8 type C domain (F5_F8_type_C)	F5/8 type C domain	This domain is also known as the discoidin (DS) domain family [1].	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A [auth C]	MURINE MONOCLONAL 3E6 FAB HEAVY CHAIN	-	-	-
B [auth D]	MURINE MONOCLONAL 3E6 FAB LIGHT CHAIN	-	-	-
C [auth E]	MURINE MONOCLONAL G99 FAB HEAVY CHAIN	-	-	-
E [auth M]	Coagulation factor VIII	oxidoreductase activity catalytic activity cation binding transition metal ion binding copper ion binding ion binding binding metal ion binding small molecule binding	multicellular organismal process regulation of body fluid levels wound healing response to stimulus hemostasis biological regulation blood coagulation response to stress coagulation regulation of biological quality response to wounding acute-phase response defense response inflammatory response multicellular organismal process regulation of body fluid levels wound healing response to stimulus hemostasis biological regulation blood coagulation response to stress coagulation regulation of biological quality response to wounding acute-phase response defense response inflammatory response acute inflammatory response protein maturation blood coagulation, intrinsic pathway blood coagulation, fibrin clot formation protein metabolic process protein activation cascade primary metabolic process gene expression macromolecule biosynthetic process biosynthetic process metabolic process cellular process macromolecule metabolic process	membrane-bounded organelle intracellular anatomical structure Golgi apparatus membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen Golgi lumen intracellular organelle cellular anatomical structure organelle lumen organelle cytoplasm endoplasmic reticulum lumen membrane-bounded organelle intracellular anatomical structure Golgi apparatus membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen Golgi lumen intracellular organelle cellular anatomical structure organelle lumen organelle cytoplasm endoplasmic reticulum lumen endoplasmic reticulum extracellular region membrane bounding membrane of organelle endoplasmic reticulum-Golgi intermediate compartment membrane endoplasmic reticulum-Golgi intermediate compartment organelle membrane vesicle lumen intracellular vesicle cytoplasmic vesicle lumen secretory granule lumen cytoplasmic vesicle secretory granule secretory vesicle platelet alpha granule vesicle platelet alpha granule lumen cell periphery plasma membrane COPII-coated ER to Golgi transport vesicle coated vesicle extracellular space
D [auth F]	MURINE MONOCLONAL G99 FAB LIGHT CHAIN	-	-	-

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
E [auth M]	IPR033138	Multicopper oxidases, conserved site	Conserved Site
E [auth M]	IPR008979	Galactose-binding-like domain superfamily	Homologous Superfamily
E [auth M]	IPR011707	Multicopper oxidase-like, N-terminal	Domain
E [auth M]	IPR024715	Coagulation factor 5/8-like	Family
E [auth M]	IPR000421	Coagulation factor 5/8, C-terminal domain	Domain
E [auth M]	IPR008972	Cupredoxin	Homologous Superfamily
E [auth M]	IPR011706	Multicopper oxidase, C-terminal	Domain
E [auth M]	IPR050633	Neuropilin/Multicopper Oxidase/Coagulation Factor	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
E [auth M]	Pharos: P00451	hemophilia A thrombophilia Venous Thrombosis autoimmune disease lung carcinoma MALT lymphoma nephrosclerosis pancreatic intraductal papillary-mucinous carcinoma acquired hemophilia primitive neuroectodermal tumor astrocytic tumor thrombotic disease ductal breast carcinoma in situ hemophilia hereditary von Willebrand disease hemophilia A thrombophilia Venous Thrombosis autoimmune disease lung carcinoma MALT lymphoma nephrosclerosis pancreatic intraductal papillary-mucinous carcinoma acquired hemophilia primitive neuroectodermal tumor astrocytic tumor thrombotic disease ductal breast carcinoma in situ hemophilia hereditary von Willebrand disease lung adenocarcinoma malignant ependymoma medulloblastoma non-small cell lung carcinoma pancreatic intraductal papillary-mucinous neoplasm acquired von willebrand syndrome severe hemophilia A atypical teratoid rhabdoid tumor tuberculosis congenital factor XI deficiency invasive ductal breast carcinoma lung cancer malignant pancreatic neoplasm mild hemophilia A pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia arthropathy vascular disease hemophilia B large cell medulloblastoma

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM157729.