6GVL

Second pair of Fibronectin type III domains of integrin beta4 bound to the bullous pemphigoid antigen BP230 (BPAG1e)

External Resource: Annotation

Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	PF00041	e6gvlA1	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: PF00041	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (utative)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF00041	Fibronectin type III domain (fn3)	Fibronectin type III domain	-	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	Integrin beta-4	cell adhesion molecule binding protein-containing complex binding protein binding binding integrin binding signaling receptor binding G protein-coupled receptor binding cation binding ion binding metal ion binding small molecule binding	cell adhesion cell-matrix adhesion cell-substrate adhesion cellular process cellular catabolic process catabolic process cellular metabolic process autophagy metabolic process process utilizing autophagic mechanism cellular response to stimulus regulation of biological process integrin-mediated signaling pathway regulation of cellular process cell adhesion cell-matrix adhesion cell-substrate adhesion cellular process cellular catabolic process catabolic process cellular metabolic process autophagy metabolic process process utilizing autophagic mechanism cellular response to stimulus regulation of biological process integrin-mediated signaling pathway regulation of cellular process cell surface receptor signaling pathway response to stimulus signaling biological regulation cell communication signal transduction multicellular organismal process cellular developmental process peripheral nervous system axon ensheathment cellular component assembly involved in morphogenesis cell development glial cell development myelin assembly cellular component organization cellular component biogenesis multicellular organism development neurogenesis cellular anatomical entity morphogenesis cellular component assembly gliogenesis axon ensheathment ensheathment of neurons cell differentiation anatomical structure development myelination in peripheral nervous system cellular component organization or biogenesis Schwann cell differentiation system development developmental process peripheral nervous system development nervous system development peripheral nervous system myelin formation myelination anatomical structure morphogenesis Schwann cell development glial cell differentiation anatomical structure formation involved in morphogenesis cell junction organization cell junction assembly cell-substrate junction assembly cell-substrate junction organization hemidesmosome assembly nail development limb development appendage development filopodium assembly plasma membrane bounded cell projection organization cell projection organization plasma membrane bounded cell projection assembly cell projection assembly response to stress response to wounding formation of primary germ layer mesodermal cell differentiation mesoderm morphogenesis embryonic morphogenesis tissue development embryo development gastrulation tissue morphogenesis mesoderm formation mesoderm development skin development skin morphogenesis animal organ development animal organ morphogenesis cell motility trophoblast cell migration multi-multicellular organism process reproductive process ameboidal-type cell migration cell migration female pregnancy embryo implantation multi-organism reproductive process	cell junction cellular anatomical entity anchoring junction focal adhesion cell-substrate junction membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen intracellular membrane-bounded organelle nucleolus intracellular organelle lumen intracellular organelle organelle lumen cell junction cellular anatomical entity anchoring junction focal adhesion cell-substrate junction membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen intracellular membrane-bounded organelle nucleolus intracellular organelle lumen intracellular organelle organelle lumen organelle intracellular non-membrane-bounded organelle nuclear lumen non-membrane-bounded organelle hemidesmosome nuclear membrane membrane endomembrane system organelle envelope nuclear envelope organelle membrane basement membrane cell periphery extracellular matrix external encapsulating structure collagen-containing extracellular matrix basal part of cell plasma membrane basal plasma membrane plasma membrane region extracellular exosome extracellular vesicle extracellular organelle extracellular region extracellular space vesicle extracellular membrane-bounded organelle cell surface cell leading edge protein-containing complex plasma membrane protein complex membrane protein complex receptor complex integrin complex plasma membrane signaling receptor complex protein complex involved in cell adhesion
B	Dystonin	cytoskeletal protein binding microtubule binding protein binding binding tubulin binding microtubule plus-end binding cation binding ion binding calcium ion binding metal ion binding small molecule binding cell adhesion molecule binding protein-containing complex binding integrin binding cytoskeletal protein binding microtubule binding protein binding binding tubulin binding microtubule plus-end binding cation binding ion binding calcium ion binding metal ion binding small molecule binding cell adhesion molecule binding protein-containing complex binding integrin binding signaling receptor binding identical protein binding protein homodimerization activity protein dimerization activity actin binding structural molecule activity	cellular component organization cellular component biogenesis cell junction organization cellular component assembly cell junction assembly cell-substrate junction assembly cell-substrate junction organization hemidesmosome assembly cellular process cellular component organization or biogenesis organelle organization cytoskeleton organization response to stimulus response to stress cellular component organization cellular component biogenesis cell junction organization cellular component assembly cell junction assembly cell-substrate junction assembly cell-substrate junction organization hemidesmosome assembly cellular process cellular component organization or biogenesis organelle organization cytoskeleton organization response to stimulus response to stress response to wounding cell adhesion intermediate filament-based process intermediate filament cytoskeleton organization cell motility cellular response to stimulus regulation of biological process integrin-mediated signaling pathway regulation of cellular process cell surface receptor signaling pathway signaling biological regulation cell communication signal transduction maintenance of cell polarity establishment or maintenance of cell polarity wound healing microtubule cytoskeleton organization microtubule-based process cellular localization transport retrograde axonal transport intracellular transport axonal transport establishment of localization localization axo-dendritic transport establishment of localization in cell microtubule-based transport transport along microtubule cytoskeleton-dependent intracellular transport microtubule-based movement	membrane cellular anatomical entity intracellular anatomical structure Z disc sarcomere intracellular organelle I band organelle intracellular non-membrane-bounded organelle contractile muscle fiber cytoplasm supramolecular fiber myofibril supramolecular polymer membrane cellular anatomical entity intracellular anatomical structure Z disc sarcomere intracellular organelle I band organelle intracellular non-membrane-bounded organelle contractile muscle fiber cytoplasm supramolecular fiber myofibril supramolecular polymer supramolecular complex non-membrane-bounded organelle membrane-bounded organelle organelle envelope nuclear envelope nucleus intracellular membrane-bounded organelle endomembrane system basement membrane cell periphery extracellular matrix external encapsulating structure collagen-containing extracellular matrix cytoskeleton actin cytoskeleton cytosol cell leading edge actin filament bundle actomyosin stress fiber contractile actin filament bundle axon cell projection neuron projection plasma membrane bounded cell projection intermediate filament cytoskeleton neuron projection cytoplasm axon cytoplasm cytoplasmic region plasma membrane bounded cell projection cytoplasm anchoring junction cell junction focal adhesion cell-substrate junction cell cortex hemidesmosome basal part of cell plasma membrane basal plasma membrane plasma membrane region polymeric cytoskeletal fiber intermediate filament microtubule end microtubule microtubule plus-end microtubule cytoskeleton membrane-enclosed lumen intracellular organelle lumen nucleoplasm organelle lumen nuclear lumen organelle subcompartment endoplasmic reticulum subcompartment nuclear outer membrane-endoplasmic reticulum membrane network endoplasmic reticulum organelle membrane endoplasmic reticulum membrane cytoplasmic vesicle intracellular vesicle vesicle H zone A band

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR033760	Integrin beta N-terminal	Domain
A	IPR013111	EGF-like domain, extracellular	Domain
A	IPR015812	Integrin beta subunit	Family
A	IPR036349	Integrin beta tail domain superfamily	Homologous Superfamily
A	IPR002369	Integrin beta subunit, VWA domain	Domain
A	IPR016201	PSI domain	Domain
A	IPR040622	Integrin beta, epidermal growth factor-like domain 1	Domain
A	IPR000742	EGF-like domain	Domain
A	IPR012896	Integrin beta subunit, tail	Domain
A	IPR003961	Fibronectin type III	Domain
A	IPR036465	von Willebrand factor A-like domain superfamily	Homologous Superfamily
A	IPR003644	Na-Ca exchanger/integrin-beta4	Domain
A	IPR013783	Immunoglobulin-like fold	Homologous Superfamily
A	IPR038081	CalX-like domain superfamily	Homologous Superfamily
A	IPR012013	Integrin beta-4 subunit	Family
A	IPR036116	Fibronectin type III superfamily	Homologous Superfamily
B	IPR041615	Desmoplakin, SH3 domain	Domain
B	IPR002048	EF-hand domain	Domain
B	IPR018247	EF-Hand 1, calcium-binding site	Binding Site
B	IPR001589	Actinin-type actin-binding domain, conserved site	Conserved Site
B	IPR049538	Plectin-like, spectrin-like repeat	Repeat
B	IPR035915	Plakin repeat superfamily	Homologous Superfamily
B	IPR003108	GAR domain	Domain
B	IPR001452	SH3 domain	Domain
B	IPR036872	CH domain superfamily	Homologous Superfamily
B	IPR041573	Desmoplakin, spectrin-like domain	Domain
B	IPR036534	GAR domain superfamily	Homologous Superfamily
B	IPR002017	Spectrin repeat	Repeat
B	IPR043197	Plakin	Family
B	IPR001101	Plectin repeat	Repeat
B	IPR001715	Calponin homology domain	Domain
B	IPR011992	EF-hand domain pair	Homologous Superfamily
B	IPR018159	Spectrin/alpha-actinin	Repeat

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P16144	junctional epidermolysis bullosa epidermolysis bullosa junctional epidermolysis bullosa with pyloric atresia non-small cell lung carcinoma epidermolysis bullosa simplex pyloric atresia junctional epidermolysis bullosa, non-Herlitz type salivary gland adenoid cystic carcinoma Gliosis localized epidermolysis bullosa simplex adenoid cystic carcinoma ectodermal dysplasia syndrome epidermolysis bullosa simplex with pyloric atresia generalized junctional epidermolysis bullosa non-Herlitz type lung adenocarcinoma junctional epidermolysis bullosa epidermolysis bullosa junctional epidermolysis bullosa with pyloric atresia non-small cell lung carcinoma epidermolysis bullosa simplex pyloric atresia junctional epidermolysis bullosa, non-Herlitz type salivary gland adenoid cystic carcinoma Gliosis localized epidermolysis bullosa simplex adenoid cystic carcinoma ectodermal dysplasia syndrome epidermolysis bullosa simplex with pyloric atresia generalized junctional epidermolysis bullosa non-Herlitz type lung adenocarcinoma malignant ependymoma aplasia cutis congenita Clouston syndrome pilocytic astrocytoma glioblastoma junctional epidermolysis bullosa inversa large cell medulloblastoma lung cancer malignant glioma amelogenesis imperfecta malignant pancreatic neoplasm astrocytic tumor osteosarcoma Down syndrome psoriasis salivary gland cancer interstitial cystitis lung carcinoma malignant mesothelioma medulloblastoma pancreatic ductal adenocarcinoma
B	Pharos: Q03001	hereditary sensory and autonomic neuropathy type 6 epidermolysis bullosa simplex due to BP230 deficiency peripheral neuropathy epidermolysis bullosa malignant ependymoma malignant pancreatic neoplasm neurodegenerative disease osteosarcoma acute myeloid leukemia peripheral nervous system disease breast cancer pituitary cancer dermatitis tuberculosis EPIDERMOLYSIS BULLOSA SIMPLEX, AUTOSOMAL RECESSIVE (disorder) hereditary sensory and autonomic neuropathy type 6 epidermolysis bullosa simplex due to BP230 deficiency peripheral neuropathy epidermolysis bullosa malignant ependymoma malignant pancreatic neoplasm neurodegenerative disease osteosarcoma acute myeloid leukemia peripheral nervous system disease breast cancer pituitary cancer dermatitis tuberculosis EPIDERMOLYSIS BULLOSA SIMPLEX, AUTOSOMAL RECESSIVE (disorder) invasive ductal breast carcinoma lung cancer malignant glioma medulloblastoma non-small cell lung carcinoma ovarian cancer astrocytic tumor breast carcinoma progressive supranuclear palsy esophageal adenocarcinoma hereditary spastic paraplegia KRT14-related epidermolysis bullosa simplex malignant colon neoplasm malignant mesothelioma nasopharyngeal carcinoma oligodendroglioma pancreatic ductal adenocarcinoma atypical teratoid rhabdoid tumor Pick disease cutaneous lupus erythematosus psoriasis glioblastoma interstitial cystitis lung adenocarcinoma