8G6I | pdb_00008g6i

Coagulation factor VIII bound to a patient-derived anti-C1 domain antibody inhibitor

External Resource: Annotation

Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
SAbDab Antibody Annotation
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	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	F_UNCLASSIFIED	e8g6iA8	A: beta sandwiches	X: jelly-roll	H: Concanavalin A-like	T: Galactose-binding domain-like	F: F_UNCLASSIFIED	ECOD (1.6)
A	F_UNCLASSIFIED	e8g6iA2	A: beta sandwiches	X: Cupredoxin-like	H: Cupredoxin-related (From Topology)	T: Cupredoxin-related	F: F_UNCLASSIFIED	ECOD (1.6)
B	F_UNCLASSIFIED	e8g6iB2	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: F_UNCLASSIFIED	ECOD (1.6)
C	F_UNCLASSIFIED	e8g6iC2	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: F_UNCLASSIFIED	ECOD (1.6)

SAbDab Antibody Annotation SAbDab Database Homepage

Chain	Chain Class	Chain Subclass	Chain Type	Antigen Name(s)	Provenance Source
B	Light Chain	IGKV1	Kappa	coagulation factor viii chimera from human and pig	SAbDab (2025-03-21)
C	Heavy Chain	IGHV3	-	coagulation factor viii chimera from human and pig	SAbDab (2025-03-21)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	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
A	PF00394	Multicopper oxidase (Cu-oxidase)	Multicopper oxidase	Many of the proteins in this family contain multiple similar copies of this plastocyanin-like domain.	Domain
A	PF07732	Multicopper oxidase (Cu-oxidase_3)	Multicopper oxidase	This entry contains many divergent copper oxidase-like domains that are not recognised by the Pfam:PF00394 model.	Domain
A	PF07731	Multicopper oxidase (Cu-oxidase_2)	Multicopper oxidase	This entry contains many divergent copper oxidase-like domains that are not recognised by the Pfam:PF00394 model.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	Coagulation factor VIII chimera from human and pig	oxidoreductase activity catalytic activity cation binding transition metal ion binding copper ion binding ion binding binding metal ion binding small molecule binding	acute-phase response response to stimulus defense response response to stress inflammatory response acute inflammatory response multicellular organismal process regulation of body fluid levels protein maturation blood coagulation, intrinsic pathway blood coagulation, fibrin clot formation coagulation protein metabolic process regulation of biological quality acute-phase response response to stimulus defense response response to stress inflammatory response acute inflammatory response multicellular organismal process regulation of body fluid levels protein maturation blood coagulation, intrinsic pathway blood coagulation, fibrin clot formation coagulation protein metabolic process regulation of biological quality response to wounding wound healing protein activation cascade hemostasis biological regulation primary metabolic process gene expression macromolecule biosynthetic process blood coagulation biosynthetic process metabolic process cellular process macromolecule metabolic process	cellular anatomical structure extracellular region extracellular space membrane-bounded organelle intracellular anatomical structure Golgi apparatus membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen Golgi lumen intracellular organelle organelle lumen organelle cellular anatomical structure extracellular region extracellular space membrane-bounded organelle intracellular anatomical structure Golgi apparatus membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen Golgi lumen intracellular organelle organelle lumen organelle cytoplasm endoplasmic reticulum lumen endoplasmic reticulum 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
B	NB33 light chain	-	-	-
C	NB33 heavy chain	-	-	-

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR001117	Multicopper oxidase, second cupredoxin domain	Domain
A	IPR033138	Multicopper oxidases, conserved site	Conserved Site
A	IPR008979	Galactose-binding-like domain superfamily	Homologous Superfamily
A	IPR011707	Multicopper oxidase-like, N-terminal	Domain
A	IPR024715	Coagulation factor 5/8-like	Family
A	IPR000421	Coagulation factor 5/8, C-terminal domain	Domain
A	IPR008972	Cupredoxin	Homologous Superfamily
A	IPR011706	Multicopper oxidase, C-terminal	Domain
A	IPR050633	Neuropilin/Multicopper Oxidase/Coagulation Factor	Family
A	IPR033138	Multicopper oxidases, conserved site	Conserved Site
A	IPR008979	Galactose-binding-like domain superfamily	Homologous Superfamily
A	IPR011707	Multicopper oxidase-like, N-terminal	Domain
A	IPR024715	Coagulation factor 5/8-like	Family
A	IPR000421	Coagulation factor 5/8, C-terminal domain	Domain
A	IPR008972	Cupredoxin	Homologous Superfamily
A	IPR011706	Multicopper oxidase, C-terminal	Domain
A	IPR050633	Neuropilin/Multicopper Oxidase/Coagulation Factor	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	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.