Fibulin (FY-beau-lin) (now known as Fibulin-1 FBLN1) is the prototypic member of a multigene family, currently with seven members. Fibulin-1 is a calcium-binding glycoprotein. In vertebrates, fibulin-1 is found in blood and extracellular matrices. In the extracellular matrix, fibulin-1 associates with basement membranes and elastic fibers. The association with these matrix structures is mediated by its ability to interact with numerous extracellular matrix constituents including fibronectin, proteoglycans, laminins and tropoelastin. In blood, fibulin-1 binds to fibrinogen and incorporates into clots.
Fibulins are secreted glycoproteins that become incorporated into a fibrillar extracellular matrix when expressed by cultured cells or added exogenously to cell monolayers.[2][3] The five known members of the family share an elongated structure and many calcium-binding sites, owing to the presence of tandem arrays of epidermal growth factor-like domains. They have overlapping binding sites for several basement-membrane proteins, tropoelastin, fibrillin, fibronectin and proteoglycans, and they participate in diverse supramolecular structures. The amino-terminal domain I of fibulin consists of three anaphylatoxin-like (AT) modules, each approximately 40 residues long and containing four or six cysteines. The structure of an AT module was determined for the complement-derived anaphylatoxin C3a, and was found to be a compact alpha-helical fold that is stabilized by three disulphide bridges in the pattern Cys14, Cys25 and Cys36 (where Cys is cysteine). The bulk of the remaining portion of the fibulin molecule is a series of nine EGF-like repeats.[4]
^Williamson MP, Madison VS (March 1990). "Three-dimensional structure of porcine C5adesArg from 1H nuclear magnetic resonance data". Biochemistry. 29 (12): 2895–905. doi:10.1021/bi00464a002. PMID 2337573.
^Burgess WH, Argraves WS, Tran H, Dickerson K (1990). "Fibulin is an extracellular matrix and plasma glycoprotein with repeated domain structure". J. Cell Biol. 111 (6): 3155–3164. doi:10.1083/jcb.111.6.3155. PMC 2116371. PMID 2269669.
^Timpl R, Sasaki T, Kostka G, Chu ML (2003). "Fibulins: a versatile family of extracellular matrix proteins". Nat. Rev. Mol. Cell Biol. 4 (6): 479–489. doi:10.1038/nrm1130. PMID 12778127. S2CID 8442153.
^Timpl R, Sasaki T, Chu ML, Pan TC, Zhang RZ, Fassler R (1993). "Structure and expression of fibulin-2, a novel extracellular matrix protein with multiple EGF-like repeats and consensus motifs for calcium binding". J. Cell Biol. 123 (5): 1269–1277. doi:10.1083/jcb.123.5.1269. PMC 2119879. PMID 8245130.
Fibulin (FY-beau-lin) (now known as Fibulin-1 FBLN1) is the prototypic member of a multigene family, currently with seven members. Fibulin-1 is a calcium-binding...
FBLN1 is the gene encoding fibulin-1, an extracellular matrix and plasma protein. Fibulin-1 is a secreted glycoprotein that is found in association with...
a highly complex process involving several key proteins including fibulin-4, fibulin-5, latent transforming growth factor β binding protein 4, and microfibril...
(September 2009). "Differential regulation of elastic fiber formation by fibulin-4 and -5". The Journal of Biological Chemistry. 284 (36): 24553–24567....
also cause hereditary angioedema. Fibulin-5 mutation: Rare forms of the disease are caused by genetic defects in fibulin-5, in an autosomal dominant manner...
membranes via several types of protein, including fibronectin, laminin, fibulin and integrin. Vitamin C deficiency causes scurvy, a serious and painful...
Iatrogenic L A ACatKe Cathepsin K Tumour associated L A AEFEMP1e EGF-containing fibulin-like extracellular matrix protein 1 (EFEMP1) Portal veins, Aging associated...
EGF-containing fibulin-like extracellular matrix protein 1 is a protein that in humans is encoded by the EFEMP1 gene. This gene encodes a member of the fibulin family...
macrophages. It has also been considered that mutations in elastin (ELN) and fibulin-5 (FBLN5) genes can increase susceptibility of elastic fibres to inflammatory...
Fibulin-5 (also known as DANCE (developmental arteries and neural crest epidermal growth factor (EGF)-like)) is a protein that in humans is encoded by...
org. Retrieved 2016-12-06. Hirai, M; Ohbayashi, T; Horiguchi, M (2007). "Fibulin-5/DANCE has an elastogenic organizer activity that is abrogated by proteolytic...
Fibulin-2 is a protein that in humans is encoded by the FBLN2 gene. This gene encodes an extracellular matrix protein, which belongs to the fibulin family...
EGF-containing fibulin-like extracellular matrix protein 2 is a protein that in humans is encoded by the EFEMP2 gene. A large number of extracellular...
structural support. Other molecular components of the basal membrane include fibulin-1, fibronectin, entactin, and heparin sulfate proteoglycan perlecan. Ultimately...
interacts with members of the TGF-β superfamily, fibronectin, perlecan, fibulin-1, slit, and mucins. Knockout mice with the Ctgf gene disrupted die at...
Mann K, Timpl R (August 2001). "Mapping of binding sites for nidogens, fibulin-2, fibronectin and heparin to different IG modules of perlecan". J. Mol...
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