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FBLN1
Identifikatori
Aliasi	FBLN1
Vanjski ID-jevi	OMIM: 135820 MGI: 95487 HomoloGene: 21295 GeneCards: FBLN1
Lokacija gena (čovjek)
Hrom.	Hromosom 22 (čovjek)
Kraj	45,601,135 bp
Lokacija gena (miš)
Hrom.	Hromosom 15 (miš)
Kraj	85,170,736 bp
Obrazac RNK ekspresije
	; ; ; ;
	Više referentnih podataka o ekspresiji
Ontologija gena
Molekularna funkcija	• fibronectin binding; • calcium ion binding; • extracellular matrix structural constituent; • fibrinogen binding; • integrin binding; • protein C-terminus binding; • GO:0001948, GO:0016582 vezivanje za proteine; • vezivanje identičnih proteina; • peptidase activator activity; • GO:0032403 protein-containing complex binding;
Ćelijska komponenta	• elastic fiber; • GO:0005578 Vanćelijski matriks; • Fibrinogen; • basement membrane; • Egzosom; • Vanćelijsko; • extracellular region; • collagen-containing extracellular matrix;
Biološki proces	• GO:0033128 negative regulation of protein phosphorylation; • negative regulation of cell adhesion; • negative regulation of stem cell proliferation; • positive regulation of fibroblast proliferation; • positive regulation of substrate-dependent cell migration, cell attachment to substrate; • negative regulation of cell motility; • negative regulation of transformation of host cell by virus; • positive regulation of peptidase activity; • GO:1901313 positive regulation of gene expression; • negative regulation of ERK1 and ERK2 cascade; • integrin-mediated signaling pathway; • blood coagulation, fibrin clot formation; • negative regulation of substrate adhesion-dependent cell spreading; • GO:0022415 viral process; • embryo implantation; • extracellular matrix organization;
	Izvori:Amigo / QuickGO
Ortolozi
	2192
	14114
	ENSG00000077942
	ENSMUSG00000006369
	P23142
	Q08879
	NM_006487; NM_001996; NM_006485; NM_006486
	NM_010180; NM_001347088
	NP_001987; NP_006476; NP_006477; NP_006478
	NP_001334017; NP_034310
	Wikipodaci
Pogledaj/uredi – čovjek	Pogledaj/uredi – miš

FBLN1 je gen koji je kod ljudi lociran na hromosomu 22. Kodira fibulin-1, vanćelijski plazmatski protein.^[5]^[6]^[7]

Aminokiselinska sekvenca

Dužina polipeptidnog lanca je 703 aminokiseline, a molekulska težina 77.214 Da.^[5]

C: Cistein

D: Asparaginska kiselina

E: Glutaminska kiselina

F: Fenilalanin

G: Glicin

H: Histidin

I: Izoleucin

K: Lizin

L: Leucin

M: Metionin

N: Asparagin

P: Prolin

Q: Glutamin

R: Arginin

S: Serin

T: Treonin

V: Valin

W: Triptofan

Y: Tirozin

10	20	30	40	50
MERAAPSRRV	PLPLLLLGGL	ALLAAGVDAD	VLLEACCADG	HRMATHQKDC
SLPYATESKE	CRMVQEQCCH	SQLEELHCAT	GISLANEQDR	CATPHGDNAS
LEATFVKRCC	HCCLLGRAAQ	AQGQSCEYSL	MVGYQCGQVF	QACCVKSQET
GDLDVGGLQE	TDKIIEVEEE	QEDPYLNDRC	RGGGPCKQQC	RDTGDEVVCS
CFVGYQLLSD	GVSCEDVNEC	ITGSHSCRLG	ESCINTVGSF	RCQRDSSCGT
GYELTEDNSC	KDIDECESGI	HNCLPDFICQ	NTLGSFRCRP	KLQCKSGFIQ
DALGNCIDIN	ECLSISAPCP	IGHTCINTEG	SYTCQKNVPN	CGRGYHLNEE
GTRCVDVDEC	APPAEPCGKG	HRCVNSPGSF	RCECKTGYYF	DGISRMCVDV
NECQRYPGRL	CGHKCENTLG	SYLCSCSVGF	RLSVDGRSCE	DINECSSSPC
SQECANVYGS	YQCYCRRGYQ	LSDVDGVTCE	DIDECALPTG	GHICSYRCIN
IPGSFQCSCP	SSGYRLAPNG	RNCQDIDECV	TGIHNCSINE	TCFNIQGGFR
CLAFECPENY	RRSAATLQQE	KTDTVRCIKS	CRPNDVTCVF	DPVHTISHTV
ISLPTFREFT	RPEEIIFLRA	ITPPHPASQA	NIIFDITEGN	LRDSFDIIKR
YMDGMTVGVV	RQVRPIVGPF	HAVLKLEMNY	VVGGVVSHRN	VVNVHIFVSE
YWF

Struktura

Fibulin-1 ima modularnu strukturu domena i uključuje seriju od devet modula sličnih epidermnom faktoru rasta praćenih modulom tipa fibulin, modul koji se nalazi u svim članovima porodice gena fibulina.^[6]

Ljudski fibulin-1 gen, FBLN1, kodira četiri prerađene varijante označene kao fibulin-1A, B, C i D, koje se razlikuju po svojim karboksi terminalnim regijama. Kod miša, kokoši i nematode, Caenorhabditis elegans, proizvode se samo dvije varijante fibulina-1: fibulin-1C i fibulin-1D.^[5]

Funkcija

Fibulin-1 je izlučeni glikoprotein koji se nalazi u vezi sa strukturama vanćelijski matriksa, uključujući vlakna koja sadrže fibronektin, vlakna koja sadrže elastin i baznu membranu . Fibulin-1 se vezuje za niz sastojaka vanćelijskog matriksa uključujući fibronektin,^[7] nidogen-1, proteoglikan i verzikan .^[7]^[8] Fibulin-1 je takođe krvni protein sposoban da se veže za fibrinogen.^[9]

Interakcije

Pokazalo se da je FBLN1 u interakciji sa:

Također pogledajte

Sinpolidaktilija

Reference

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000077942 - Ensembl, maj 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000006369 - Ensembl, maj 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ ^a ^b ^c "Entrez Gene: FBLN1 fibulin 1".
^ ^a ^b 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.
^ ^a ^b ^c 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. doi:10.1016/S0021-9258(19)88674-X. PMID 1400330. Arhivirano s originala, 30. 6. 2008. Pristupljeno 6. 2. 2022.
^ 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. S2CID 8442153.
^ ^a ^b 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. S2CID 13501440.
^ 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. Bibcode:1999PNAS...96..869P. doi:10.1073/pnas.96.3.869. PMC 15317. PMID 9927660.
^ 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.
^ 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.
^ 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.
^ 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. 215 (3): 733–40. doi:10.1111/j.1432-1033.1993.tb18086.x. PMID 8354280.
^ 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. doi:10.1016/S0021-9258(19)88631-3. PMID 1400300.

Dopunska literatura

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. doi:10.1016/S0021-9258(19)88674-X. 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. S2CID 24834825.
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. doi:10.1242/jcs.107.1.329. 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. 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. Bibcode:1996PNAS...93..316C. 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. S2CID 25996538.
Godyna S, Diaz-Ricart M, Argraves WS (Oct 1996). "Fibulin-1 mediates platelet adhesion via a bridge of fibrinogen". Blood. 88 (7): 2569–77. doi:10.1182/blood.V88.7.2569.bloodjournal8872569. 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. 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. Bibcode:1999PNAS...96..869P. doi:10.1073/pnas.96.3.869. PMC 15317. PMID 9927660.

Vanjski linkovi

[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000077942 - Ensembl, maj 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000006369 - Ensembl, maj 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[entrez-5] "Entrez Gene: FBLN1 fibulin 1".

[pmid2269669-6] 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.

[pmid1400330-7] 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. doi:10.1016/S0021-9258(19)88674-X. PMID 1400330. Arhivirano s originala, 30. 6. 2008. Pristupljeno 6. 2. 2022.

[pmid12778127-8] 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. S2CID 8442153.

[pmid19693531-9] 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. S2CID 13501440.

[pmid9927660-10] 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. Bibcode:1999PNAS...96..869P. doi:10.1073/pnas.96.3.869. PMC 15317. PMID 9927660.

[pmid11238726-11] 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.

[pmid9299350-12] 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.

[pmid9278415-13] 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.

[pmid8354280-14] 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. 215 (3): 733–40. doi:10.1111/j.1432-1033.1993.tb18086.x. PMID 8354280.

[pmid1400300-15] 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. doi:10.1016/S0021-9258(19)88631-3. PMID 1400300.

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