9D5D | pdb_00009d5d

Crystal Structure of Blood Coagulation Factor VIII C2 Domain Mutant L2251A/L2252A

External Resource: Annotation

Domain Annotation: SCOP2 Classification
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 [auth M]	SCOP2B Superfamily	galactose-binding domain-like	8041137	3000087	SCOP2B (2022-06-29)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A [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 M]	Factor VIIIa light chain	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

InterPro: Protein Family Classification InterPro Database Homepage

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

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A [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.