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Showing Protein EGF-containing fibulin-like extracellular matrix protein 2 (HMDBP13403)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 1 metabolite
Identification
HMDB Protein ID HMDBP13403
Secondary Accession Numbers None
Name EGF-containing fibulin-like extracellular matrix protein 2
Synonyms
  1. Fibulin-4
  2. Protein UPH1
  3. FIBL-4
Gene Name EFEMP2
Protein Type Unknown
Biological Properties
General Function Not Available
Specific Function Plays a crucial role in elastic fiber formation in tissue, and in the formation of ultrastructural connections between elastic laminae and smooth muscle cells in the aorta, therefore participates in terminal differentiation and maturation of smooth muscle cell (SMC) and in the mechanical properties and wall integrity maintenance of the aorta (PubMed:27339457). In addition, is involved in the control of collagen fibril assembly in tissue throught proteolytic activation of LOX leading to cross- linking of collagen and elastin (By similarity). Also promotes ELN coacervation and participates in the deposition of ELN coacervates on to microfibrils but also regulates ELN cross- linking through LOX interaction (PubMed:18973305, PubMed:19570982). Moreover adheres to the cells through heparin binding in a calcium-dependent manner and regulates vascularlar smooth muscle cells proliferation through angiotensin signaling (PubMed:23782690).
Pathways Not Available
Reactions Not Available
GO Classification
Biological Process
vascular associated smooth muscle cell development
negative regulation of vascular associated smooth muscle cell proliferation
aorta smooth muscle tissue morphogenesis
positive regulation of aortic smooth muscle cell differentiation
positive regulation of smooth muscle cell-matrix adhesion
regulation of collagen fibril organization
aorta development
positive regulation of collagen fibril organization
elastic fiber assembly
Cellular Component
extracellular vesicular exosome
microfibril
basement membrane
elastic fiber
extracellular region
collagen-containing extracellular matrix
extracellular matrix
extracellular vesicle
Molecular Function
extracellular matrix structural constituent
protein homodimerization activity
calcium ion binding
heparin binding
Cellular Location Not Available
Gene Properties
Chromosome Location Not Available
Locus Not Available
SNPs Not Available
Gene Sequence Not Available
Protein Properties
Number of Residues 443
Molecular Weight 49404.97
Theoretical pI 4.938
Pfam Domain Function
Signals
  • 1-23;
Transmembrane Regions Not Available
Protein Sequence Not Available
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID O95967
UniProtKB/Swiss-Prot Entry Name FBLN4_HUMAN
PDB IDs
GenBank Gene ID Not Available
GeneCard ID Not Available
GenAtlas ID Not Available
HGNC ID Not Available
References
General References
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  3. Taylor TD, Noguchi H, Totoki Y, Toyoda A, Kuroki Y, Dewar K, Lloyd C, Itoh T, Takeda T, Kim DW, She X, Barlow KF, Bloom T, Bruford E, Chang JL, Cuomo CA, Eichler E, FitzGerald MG, Jaffe DB, LaButti K, Nicol R, Park HS, Seaman C, Sougnez C, Yang X, Zimmer AR, Zody MC, Birren BW, Nusbaum C, Fujiyama A, Hattori M, Rogers J, Lander ES, Sakaki Y: Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature. 2006 Mar 23;440(7083):497-500. [PubMed:16554811 ]
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  6. Bian Y, Song C, Cheng K, Dong M, Wang F, Huang J, Sun D, Wang L, Ye M, Zou H: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J Proteomics. 2014 Jan 16;96:253-62. doi: 10.1016/j.jprot.2013.11.014. Epub 2013 Nov 22. [PubMed:24275569 ]
  7. El-Hallous E, Sasaki T, Hubmacher D, Getie M, Tiedemann K, Brinckmann J, Batge B, Davis EC, Reinhardt DP: Fibrillin-1 interactions with fibulins depend on the first hybrid domain and provide an adaptor function to tropoelastin. J Biol Chem. 2007 Mar 23;282(12):8935-46. doi: 10.1074/jbc.M608204200. Epub 2007 Jan 25. [PubMed:17255108 ]
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  11. Djokic J, Fagotto-Kaufmann C, Bartels R, Nelea V, Reinhardt DP: Fibulin-3, -4, and -5 are highly susceptible to proteolysis, interact with cells and heparin, and form multimers. J Biol Chem. 2013 Aug 2;288(31):22821-35. doi: 10.1074/jbc.M112.439158. Epub 2013 Jun 19. [PubMed:23782690 ]
  12. Sasaki T, Hanisch FG, Deutzmann R, Sakai LY, Sakuma T, Miyamoto T, Yamamoto T, Hannappel E, Chu ML, Lanig H, von der Mark K: Functional consequence of fibulin-4 missense mutations associated with vascular and skeletal abnormalities and cutis laxa. Matrix Biol. 2016 Dec;56:132-149. doi: 10.1016/j.matbio.2016.06.003. Epub 2016 Jun 23. [PubMed:27339457 ]
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