FBLN1

Fibulin 1
Identifiers
Symbols	FBLN1 ; FBLN; FIBL1
External IDs	OMIM: 135820 MGI: 95487 HomoloGene: 21295 GeneCards: FBLN1 Gene
Gene ontology Molecular function • fibronectin binding • integrin binding • extracellular matrix structural constituent • calcium ion binding • protein binding • protein C-terminus binding • peptidase activator activity • protein complex binding • identical protein binding • fibrinogen binding Cellular component • extracellular region • fibrinogen complex • proteinaceous extracellular matrix • basement membrane • extracellular space • extracellular matrix • extracellular exosome • elastic fiber Biological process • negative regulation of protein phosphorylation • negative regulation of cell adhesion • integrin-mediated signaling pathway • embryo implantation • positive regulation of gene expression • positive regulation of peptidase activity • viral process • extracellular matrix organization • positive regulation of fibroblast proliferation • negative regulation of ERK1 and ERK2 cascade • blood coagulation, fibrin clot formation • negative regulation of substrate adhesion-dependent cell spreading • negative regulation of transformation of host cell by virus • positive regulation of substrate-dependent cell migration, cell attachment to substrate • negative regulation of cell motility • negative regulation of stem cell proliferation • negative regulation of transforming growth factor-beta secretion Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	2192	14114
Ensembl	ENSG00000077942	ENSMUSG00000006369
UniProt	P23142	Q08879
RefSeq (mRNA)	NM_001996	NM_010180
RefSeq (protein)	NP_001987	NP_034310
Location (UCSC)	Chr 22: 45.5 – 45.6 Mb	Chr 15: 85.21 – 85.29 Mb
PubMed search

FBLN1 is the gene encoding fibulin-1, an extracellular matrix and plasma protein.^[1][2][3]

Function

Fibulin-1 is a secreted glycoprotein that is found in association with extracellular matrix structures including fibronectin-containing fibers, elastin-containing fibers and basement membranes. Fibulin-1 binds to a number of extracellular matrix constituents including fibronectin,^[3] nidogen-1, and the proteoglycan, versican.^[3][4] Fibulin-1 is also a blood protein capable of binding to fibrinogen.^[5]

Structure

Fibulin-1 has modular domain structure and includes a series of nine epidermal growth factor-like modules followed by a fibulin-type module, a module found in all members of the fibulin gene family.^[2]

The human fibulin-1 gene, FBLN1, encodes four splice variants designated fibulin-1A, B, C and D, which differ in their carboxy terminal regions. In mouse, chicken and the nematode, C. elegans, only two fibulin-1 variants are produced, fibulin-1C and fibulin-1D.^[1]

Interactions

FBLN1 has been shown to interact with:

• NOV/CCN3,^[6]
• amyloid precursor protein,^[7]
• entactin,^[8][9][10]
• fibrinogen,^[5] and
• fibronectin.^[11]

See also

• Synpolydactyly

References

1. "Entrez Gene: FBLN1 fibulin 1".
2. Argraves WS, Tran H, Burgess WH, Dickerson K (Dec 1990). "Fibulin is an extracellular matrix and plasma glycoprotein with repeated domain structure". The Journal of Cell Biology 111 (6 Pt 2): 3155–64. doi:10.1083/jcb.111.6.3155. PMC 2116371. PMID 2269669.
3. Balbona K, Tran H, Godyna S, Ingham KC, Strickland DK, Argraves WS (Oct 1992). "Fibulin binds to itself and to the carboxyl-terminal heparin-binding region of fibronectin". The Journal of Biological Chemistry 267 (28): 20120–5. PMID 1400330.
4. Timpl R, Sasaki T, Kostka G, Chu ML (Jun 2003). "Fibulins: a versatile family of extracellular matrix proteins". Nature Reviews Molecular Cell Biology 4 (6): 479–89. doi:10.1038/nrm1130. PMID 12778127.
5. Argraves WS, Tanaka A, Smith EP, Twal WO, Argraves KM, Fan D, Haudenschild CC (Nov 2009). "Fibulin-1 and fibrinogen in human atherosclerotic lesions". Histochemistry and Cell Biology 132 (5): 559–65. doi:10.1007/s00418-009-0628-7. PMID 19693531.
6. Perbal B, Martinerie C, Sainson R, Werner M, He B, Roizman B (Feb 1999). "The C-terminal domain of the regulatory protein NOVH is sufficient to promote interaction with fibulin 1C: a clue for a role of NOVH in cell-adhesion signaling". Proceedings of the National Academy of Sciences of the United States of America 96 (3): 869–74. doi:10.1073/pnas.96.3.869. PMC 15317. PMID 9927660.
7. Ohsawa I, Takamura C, Kohsaka S (Mar 2001). "Fibulin-1 binds the amino-terminal head of beta-amyloid precursor protein and modulates its physiological function". Journal of Neurochemistry 76 (5): 1411–20. doi:10.1046/j.1471-4159.2001.00144.x. PMID 11238726.
8. Adam S, Göhring W, Wiedemann H, Chu ML, Timpl R, Kostka G (Sep 1997). "Binding of fibulin-1 to nidogen depends on its C-terminal globular domain and a specific array of calcium-binding epidermal growth factor-like (EG) modules". Journal of Molecular Biology 272 (2): 226–36. doi:10.1006/jmbi.1997.1244. PMID 9299350.
9. Tran H, VanDusen WJ, Argraves WS (Sep 1997). "The self-association and fibronectin-binding sites of fibulin-1 map to calcium-binding epidermal growth factor-like domains". The Journal of Biological Chemistry 272 (36): 22600–6. doi:10.1074/jbc.272.36.22600. PMID 9278415.
10. Pan TC, Kluge M, Zhang RZ, Mayer U, Timpl R, Chu ML (Aug 1993). "Sequence of extracellular mouse protein BM-90/fibulin and its calcium-dependent binding to other basement-membrane ligands". European Journal of Biochemistry / FEBS 215 (3): 733–40. doi:10.1111/j.1432-1033.1993.tb18086.x. PMID 8354280.
11. Deeney JT, Tornheim K, Korchak HM, Prentki M, Corkey BE (Oct 1992). "Acyl-CoA esters modulate intracellular Ca2+ handling by permeabilized clonal pancreatic beta-cells". The Journal of Biological Chemistry 267 (28): 19840–5. PMID 1400300.

Further reading

• Balbona K, Tran H, Godyna S, Ingham KC, Strickland DK, Argraves WS (Oct 1992). "Fibulin binds to itself and to the carboxyl-terminal heparin-binding region of fibronectin". The Journal of Biological Chemistry 267 (28): 20120–5. PMID 1400330.
• Argraves WS, Tran H, Burgess WH, Dickerson K (Dec 1990). "Fibulin is an extracellular matrix and plasma glycoprotein with repeated domain structure". The Journal of Cell Biology 111 (6 Pt 2): 3155–64. doi:10.1083/jcb.111.6.3155. PMC 2116371. PMID 2269669.
• Argraves WS, Dickerson K, Burgess WH, Ruoslahti E (Aug 1989). "Fibulin, a novel protein that interacts with the fibronectin receptor beta subunit cytoplasmic domain". Cell 58 (4): 623–9. doi:10.1016/0092-8674(89)90097-4. PMID 2527614.
• Sasaki T, Göhring W, Pan TC, Chu ML, Timpl R (Dec 1995). "Binding of mouse and human fibulin-2 to extracellular matrix ligands". Journal of Molecular Biology 254 (5): 892–9. doi:10.1006/jmbi.1995.0664. PMID 7500359.
• Roark EF, Keene DR, Haudenschild CC, Godyna S, Little CD, Argraves WS (Apr 1995). "The association of human fibulin-1 with elastic fibers: an immunohistological, ultrastructural, and RNA study". The Journal of Histochemistry and Cytochemistry 43 (4): 401–11. doi:10.1177/43.4.7534784. PMID 7534784.
• Tran H, Tanaka A, Litvinovich SV, Medved LV, Haudenschild CC, Argraves WS (Aug 1995). "The interaction of fibulin-1 with fibrinogen. A potential role in hemostasis and thrombosis". The Journal of Biological Chemistry 270 (33): 19458–64. doi:10.1074/jbc.270.33.19458. PMID 7642629.
• Mattei MG, Pan TC, Zhang RZ, Timpl R, Chu ML (Jul 1994). "The fibulin-1 gene (FBLN1) is located on human chromosome 22 and on mouse chromosome 15". Genomics 22 (2): 437–8. doi:10.1006/geno.1994.1406. PMID 7806231.
• Korenberg JR, Chen XN, Tran H, Argraves WS (1995). "Localization of the human gene for fibulin-1 (FBLN1) to chromosome band 22q13.3". Cytogenetics and Cell Genetics 68 (3-4): 192–3. doi:10.1159/000133911. PMID 7842734.
• Brown JC, Wiedemann H, Timpl R (Jan 1994). "Protein binding and cell adhesion properties of two laminin isoforms (AmB1eB2e, AmB1sB2e) from human placenta". Journal of Cell Science 107 (1): 329–38. PMID 8175920.
• Pan TC, Kluge M, Zhang RZ, Mayer U, Timpl R, Chu ML (Aug 1993). "Sequence of extracellular mouse protein BM-90/fibulin and its calcium-dependent binding to other basement-membrane ligands". European Journal of Biochemistry / FEBS 215 (3): 733–40. doi:10.1111/j.1432-1033.1993.tb18086.x. PMID 8354280.
• Clinton GM, Rougeot C, Derancourt J, Roger P, Defrenne A, Godyna S, Argraves WS, Rochefort H (Jan 1996). "Estrogens increase the expression of fibulin-1, an extracellular matrix protein secreted by human ovarian cancer cells". Proceedings of the National Academy of Sciences of the United States of America 93 (1): 316–20. doi:10.1073/pnas.93.1.316. PMC 40229. PMID 8552629.
• Miosge N, Götz W, Sasaki T, Chu ML, Timpl R, Herken R (Feb 1996). "The extracellular matrix proteins fibulin-1 and fibulin-2 in the early human embryo". The Histochemical Journal 28 (2): 109–16. doi:10.1007/BF02331415. PMID 8737292.
• Godyna S, Diaz-Ricart M, Argraves WS (Oct 1996). "Fibulin-1 mediates platelet adhesion via a bridge of fibrinogen". Blood 88 (7): 2569–77. PMID 8839849.
• Tran H, Mattei M, Godyna S, Argraves WS (Mar 1997). "Human fibulin-1D: molecular cloning, expression and similarity with S1-5 protein, a new member of the fibulin gene family". Matrix Biology 15 (7): 479–93. doi:10.1016/S0945-053X(97)90021-4. PMID 9106159.
• Tran H, VanDusen WJ, Argraves WS (Sep 1997). "The self-association and fibronectin-binding sites of fibulin-1 map to calcium-binding epidermal growth factor-like domains". The Journal of Biological Chemistry 272 (36): 22600–6. doi:10.1074/jbc.272.36.22600. PMID 9278415.
• Adam S, Göhring W, Wiedemann H, Chu ML, Timpl R, Kostka G (Sep 1997). "Binding of fibulin-1 to nidogen depends on its C-terminal globular domain and a specific array of calcium-binding epidermal growth factor-like (EG) modules". Journal of Molecular Biology 272 (2): 226–36. doi:10.1006/jmbi.1997.1244. PMID 9299350.
• Qing J, Maher VM, Tran H, Argraves WS, Dunstan RW, McCormick JJ (Oct 1997). "Suppression of anchorage-independent growth and matrigel invasion and delayed tumor formation by elevated expression of fibulin-1D in human fibrosarcoma-derived cell lines". Oncogene 15 (18): 2159–68. doi:10.1038/sj.onc.1201385. PMID 9393974.
• Hayashido Y, Lucas A, Rougeot C, Godyna S, Argraves WS, Rochefort H (Feb 1998). "Estradiol and fibulin-1 inhibit motility of human ovarian- and breast-cancer cells induced by fibronectin". International Journal of Cancer. Journal International Du Cancer 75 (4): 654–8. doi:10.1002/(SICI)1097-0215(19980209)75:4<654::AID-IJC26>3.0.CO;2-7. PMID 9466671.
• Roger P, Pujol P, Lucas A, Baldet P, Rochefort H (Nov 1998). "Increased immunostaining of fibulin-1, an estrogen-regulated protein in the stroma of human ovarian epithelial tumors". The American Journal of Pathology 153 (5): 1579–88. doi:10.1016/S0002-9440(10)65746-X. PMC 1853396. PMID 9811350.
• Perbal B, Martinerie C, Sainson R, Werner M, He B, Roizman B (Feb 1999). "The C-terminal domain of the regulatory protein NOVH is sufficient to promote interaction with fibulin 1C: a clue for a role of NOVH in cell-adhesion signaling". Proceedings of the National Academy of Sciences of the United States of America 96 (3): 869–74. doi:10.1073/pnas.96.3.869. PMC 15317. PMID 9927660.