Fibrillin

Not to be confused with Fibrin.
fibrillin 1
Crystallographic structure of the cbEGF9-hybrid2-cbEGF10 region of human fibrillin 1.[1]
Identifiers
SymbolFBN1
Alt. symbolsFBN, MFS1, WMS
NCBI gene2200
HGNC3603
OMIM134797
PDB2W86
RefSeqNM_000138
UniProtP35555
Other data
LocusChr. 15 q21.1
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StructuresSwiss-model
DomainsInterPro
fibrillin 2
Identifiers
SymbolFBN2
Alt. symbolsCCA
NCBI gene2201
HGNC3604
OMIM121050
RefSeqNM_001999
UniProtP35556
Other data
LocusChr. 5 q23-q31
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fibrillin 3
Identifiers
SymbolFBN3
NCBI gene84467
HGNC18794
OMIM608529
RefSeqNM_032447
UniProtQ75N90
Other data
LocusChr. 19 p13
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Fibrillin is a glycoprotein, which is essential for the formation of elastic fibers found in connective tissue.[2] Fibrillin is secreted into the extracellular matrix by fibroblasts and becomes incorporated into the insoluble microfibrils, which appear to provide a scaffold for deposition of elastin.[3]

  1. ^ PDB: 2W86​; Jensen SA, Iqbal S, Lowe ED, Redfield C, Handford PA (May 2009). "Structure and interdomain interactions of a hybrid domain: a disulphide-rich module of the fibrillin/LTBP superfamily of matrix proteins". Structure. 17 (5): 759–68. doi:10.1016/j.str.2009.03.014. PMC 2724076. PMID 19446531.
  2. ^ Kielty CM, Baldock C, Lee D, Rock MJ, Ashworth JL, Shuttleworth CA (February 2002). "Fibrillin: from microfibril assembly to biomechanical function". Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 357 (1418): 207–17. doi:10.1098/rstb.2001.1029. PMC 1692929. PMID 11911778.
  3. ^ Singh (2006). Textbook of human histology. Jaypee Brothers Publishers. pp. 64–. ISBN 978-81-8061-809-3. Retrieved 9 December 2010.[permanent dead link‍]