1UZJ

Integrin binding cbEGF22-TB4-cbEGF33 fragment of human fibrillin-1, holo form.

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
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	d1uzja1	Small proteins	Knottins (small inhibitors, toxins, lectins)	EGF/Laminin	EGF-type module	Fibrillin-1	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d1uzja3	Small proteins	TB module/8-cys domain	TB module/8-cys domain	TB module/8-cys domain	Fibrillin	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1uzjb1	Small proteins	Knottins (small inhibitors, toxins, lectins)	EGF/Laminin	EGF-type module	Fibrillin-1	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1uzjb3	Small proteins	TB module/8-cys domain	TB module/8-cys domain	TB module/8-cys domain	Fibrillin	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
C	d1uzjc1	Small proteins	Knottins (small inhibitors, toxins, lectins)	EGF/Laminin	EGF-type module	Fibrillin-1	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
C	d1uzjc3	Small proteins	TB module/8-cys domain	TB module/8-cys domain	TB module/8-cys domain	Fibrillin	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2 Family	EGF-type module	8022948	4003198	SCOP2 (2022-06-29)
A	SCOP2 Family	EGF-type module	8022950	4003198	SCOP2 (2022-06-29)
A	SCOP2 Family	TB module/8-cys domain	8022952	4003506	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	EGF/Laminin-like	8035328	3000412	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	EGF/Laminin-like	8035330	3000412	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	TB module/8-cys domain	8035332	3000962	SCOP2 (2022-06-29)
B	SCOP2B Superfamily	EGF/Laminin-like	8035328	3000412	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	EGF/Laminin-like	8035330	3000412	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	TB module/8-cys domain	8035332	3000962	SCOP2B (2022-06-29)
C	SCOP2B Superfamily	EGF/Laminin-like	8035330	3000412	SCOP2B (2022-06-29)
C	SCOP2B Superfamily	EGF/Laminin-like	8035328	3000412	SCOP2B (2022-06-29)
C	SCOP2B Superfamily	TB module/8-cys domain	8035332	3000962	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	PF00683	e1uzjA1	A: few secondary structure elements	X: TB module/8-cys domain (From Topology)	H: TB module/8-cys domain (From Topology)	T: TB module/8-cys domain	F: PF00683	ECOD (1.6)
A	PF07645	e1uzjA2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: PF07645	ECOD (1.6)
B	PF00683	e1uzjB1	A: few secondary structure elements	X: TB module/8-cys domain (From Topology)	H: TB module/8-cys domain (From Topology)	T: TB module/8-cys domain	F: PF00683	ECOD (1.6)
B	PF07645	e1uzjB2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: PF07645	ECOD (1.6)
C	PF00683	e1uzjC1	A: few secondary structure elements	X: TB module/8-cys domain (From Topology)	H: TB module/8-cys domain (From Topology)	T: TB module/8-cys domain	F: PF00683	ECOD (1.6)
C	PF07645	e1uzjC2	A: few secondary structure elements	X: EGF-like	H: EGF-related	T: EGF/Laminin	F: PF07645	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	2.10.25.10	Mainly Beta	Ribbon	Laminin	Laminin	CATH (4.3.0)
A	3.90.290.10	Alpha Beta	Alpha-Beta Complex	Extracellular Matrix Fibrillin	TGF-beta binding (TB) domain	CATH (4.3.0)
B	2.10.25.10	Mainly Beta	Ribbon	Laminin	Laminin	CATH (4.3.0)
B	3.90.290.10	Alpha Beta	Alpha-Beta Complex	Extracellular Matrix Fibrillin	TGF-beta binding (TB) domain	CATH (4.3.0)
C	2.10.25.10	Mainly Beta	Ribbon	Laminin	Laminin	CATH (4.3.0)
C	3.90.290.10	Alpha Beta	Alpha-Beta Complex	Extracellular Matrix Fibrillin	TGF-beta binding (TB) domain	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B, C	PF07645	Calcium-binding EGF domain (EGF_CA)	Calcium-binding EGF domain	-	Domain
A, B, C	PF00683	TB domain (TB)	TB domain	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B, C	FIBRILLIN-1	cation binding ion binding binding calcium ion binding metal ion binding small molecule binding structural molecule activity extracellular matrix structural constituent extracellular matrix constituent conferring elasticity structural molecule activity conferring elasticity cell adhesion molecule binding protein-containing complex binding protein binding integrin binding cation binding ion binding binding calcium ion binding metal ion binding small molecule binding structural molecule activity extracellular matrix structural constituent extracellular matrix constituent conferring elasticity structural molecule activity conferring elasticity cell adhesion molecule binding protein-containing complex binding protein binding integrin binding signaling receptor binding hormone activity molecular function activator activity molecular function regulator activity signaling receptor regulator activity receptor ligand activity signaling receptor activator activity heparin binding glycosaminoglycan binding carbohydrate derivative binding sulfur compound binding identical protein binding	cellular response to stimulus cellular response to nitrogen compound response to organic substance response to stimulus response to endogenous stimulus response to organonitrogen compound response to peptide hormone cellular response to peptide hormone stimulus response to oxygen-containing compound response to nitrogen compound cellular response to oxygen-containing compound cellular response to endogenous stimulus cellular response to chemical stimulus cellular response to organonitrogen compound response to hormone cellular response to stimulus cellular response to nitrogen compound response to organic substance response to stimulus response to endogenous stimulus response to organonitrogen compound response to peptide hormone cellular response to peptide hormone stimulus response to oxygen-containing compound response to nitrogen compound cellular response to oxygen-containing compound cellular response to endogenous stimulus cellular response to chemical stimulus cellular response to organonitrogen compound response to hormone response to chemical cellular process cellular response to organic substance cellular response to insulin-like growth factor stimulus cellular response to hormone stimulus developmental process heart development multicellular organism development multicellular organismal process anatomical structure development animal organ development circulatory system development system development protein localization negative regulation of transforming growth factor beta receptor signaling pathway regulation of cellular process cell surface receptor protein serine/threonine kinase signaling pathway cellular response to transforming growth factor beta stimulus negative regulation of biological process regulation of signal transduction cell communication localization cellular macromolecule localization sequestering of TGFbeta in extracellular matrix signal transduction regulation of biological process regulation of cellular response to transforming growth factor beta stimulus regulation of cellular response to growth factor stimulus maintenance of location response to transforming growth factor beta regulation of response to stimulus maintenance of protein location in extracellular region negative regulation of transmembrane receptor protein serine/threonine kinase signaling pathway sequestering of extracellular ligand from receptor extracellular regulation of signal transduction maintenance of protein location cellular localization cellular response to growth factor stimulus negative regulation of signaling signaling regulation of transmembrane receptor protein serine/threonine kinase signaling pathway negative regulation of cell communication protein localization to extracellular region regulation of transforming growth factor beta receptor signaling pathway extracellular negative regulation of signal transduction negative regulation of cellular process enzyme-linked receptor protein signaling pathway regulation of signaling cell surface receptor signaling pathway negative regulation of response to stimulus biological regulation negative regulation of signal transduction transforming growth factor beta receptor signaling pathway macromolecule localization regulation of cell communication response to growth factor transforming growth factor beta receptor superfamily signaling pathway sensory organ development eye development visual system development camera-type eye development sensory system development skeletal system development regulation of leukocyte differentiation regulation of cell differentiation regulation of cell development cell differentiation regulation of immune system process negative regulation of hemopoiesis regulation of osteoclast development negative regulation of leukocyte differentiation regulation of myeloid leukocyte differentiation negative regulation of myeloid cell differentiation negative regulation of myeloid leukocyte differentiation osteoclast development negative regulation of multicellular organismal process leukocyte differentiation myeloid leukocyte differentiation cellular developmental process regulation of developmental process cell development regulation of osteoclast differentiation osteoclast differentiation bone cell development regulation of multicellular organismal process negative regulation of cell differentiation bone development regulation of multicellular organismal development hemopoiesis regulation of myeloid cell differentiation negative regulation of developmental process negative regulation of osteoclast differentiation negative regulation of cell development negative regulation of osteoclast development myeloid cell differentiation negative regulation of immune system process immune system process myeloid cell development regulation of hemopoiesis anatomical structure morphogenesis cell adhesion cell adhesion mediated by integrin post-embryonic eye morphogenesis eye morphogenesis sensory organ morphogenesis animal organ morphogenesis metanephros development kidney development renal system development embryonic organ morphogenesis embryonic eye morphogenesis embryonic morphogenesis embryo development embryonic organ development cellular response to BMP stimulus regulation of BMP signaling pathway response to BMP sequestering of BMP in extracellular matrix negative regulation of cellular response to growth factor stimulus negative regulation of BMP signaling pathway BMP signaling pathway	basement membrane cell periphery extracellular matrix external encapsulating structure collagen-containing extracellular matrix cellular anatomical entity microfibril supramolecular fiber supramolecular polymer supramolecular complex membrane-bounded organelle intracellular anatomical structure membrane-enclosed lumen intracellular membrane-bounded organelle basement membrane cell periphery extracellular matrix external encapsulating structure collagen-containing extracellular matrix cellular anatomical entity microfibril supramolecular fiber supramolecular polymer supramolecular complex membrane-bounded organelle intracellular anatomical structure membrane-enclosed lumen intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen endoplasmic reticulum lumen intracellular organelle organelle lumen organelle endoplasmic reticulum cytoplasm extracellular region extracellular space

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B, C	IPR024731	EGF domain	Domain
A, B, C	IPR018097	EGF-like calcium-binding, conserved site	Conserved Site
A, B, C	IPR026823	Complement Clr-like EGF domain	Domain
A, B, C	IPR013032	EGF-like, conserved site	Conserved Site
A, B, C	IPR000742	EGF-like domain	Domain
A, B, C	IPR036773	TGF-beta binding (TB) domain superfamily	Homologous Superfamily
A, B, C	IPR049388	Fibrillin, first EGF domain	Domain
A, B, C	IPR009030	Growth factor receptor cysteine-rich domain superfamily	Homologous Superfamily
A, B, C	IPR001881	EGF-like calcium-binding domain	Domain
A, B, C	IPR017878	TB domain	Domain
A, B, C	IPR000152	EGF-type aspartate/asparagine hydroxylation site	PTM
A, B, C	IPR040872	Fibrillin 1, unique N-terminal domain	Domain

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B, C	Pharos: P35555	Marfan syndrome Weill-Marchesani syndrome thoracic aortic aneurysm acromicric dysplasia MASS syndrome stiff skin syndrome aortic aneurysm isolated ectopia lentis ectopia lentis 1, isolated, autosomal dominant Geleophysic dysplasia 2 geleophysic dysplasia progeroid and marfanoid aspect-lipodystrophy syndrome hyperinsulinism Ehlers-Danlos syndrome endometriosis Marfan syndrome Weill-Marchesani syndrome thoracic aortic aneurysm acromicric dysplasia MASS syndrome stiff skin syndrome aortic aneurysm isolated ectopia lentis ectopia lentis 1, isolated, autosomal dominant Geleophysic dysplasia 2 geleophysic dysplasia progeroid and marfanoid aspect-lipodystrophy syndrome hyperinsulinism Ehlers-Danlos syndrome endometriosis Shprintzen-Goldberg syndrome hyperglycemia cirrhosis of liver Liver Cirrhosis, Experimental glaucoma-ectopia-microspherophakia-stiff joints-short stature syndrome Loeys-Dietz syndrome craniosynostosis malignant mesothelioma ductal breast carcinoma in situ neonatal Marfan syndrome facioscapulohumeral muscular dystrophy ovarian cancer abdominal aortic aneurysm pilocytic astrocytoma adenoma psoriasis glioblastoma atopic eczema ulcerative colitis brain aneurysm invasive ductal breast carcinoma cerebrovascular disorder limb-girdle muscular dystrophy lung carcinoma diabetes mellitus malignant pancreatic neoplasm medulloblastoma osteogenesis imperfecta pancreatic ductal adenocarcinoma gastric carcinoma pituitary cancer subependymal giant cell astrocytoma atypical teratoid rhabdoid tumor intellectual disability breast cancer congenital contractural arachnodactyly malignant ependymoma Duchenne muscular dystrophy nasopharyngeal carcinoma osteosarcoma familial thoracic aortic aneurysm and aortic dissection pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia acute myeloid leukemia Sjogren syndrome Becker muscular dystrophy interstitial cystitis cataract juvenile dermatomyositis

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 R01GM133198.