Search Results

Overview

Uniprot IDP35555
Protein NameFibrillin-1
Gene NameFBN1
OrganismHomo sapiens

Kla Sites from experimental identification

Position Flanking peptide
2064 RMSYCYAKFEGGKCS
2360 SNRNPVTKSECCCDG
2388 QGTVAFKKLCPHGRG
666 STCYGGYKRGQCIKP
672 YKRGQCIKPLFGAVT
747 ENLRGTYKCICNSGY
799 CPKGFIYKPDLKTCE
904 GKGYSRIKGTQCEDI

Function

Structural component of the 10-12 nm diameter microfibrils of the extracellular matrix, which conveys both structural and regulatory properties to load-bearing connective tissues (PubMed:15062093, PubMed:1860873). Fibrillin-1-containing microfibrils provide long-term force bearing structural support (PubMed:27026396). In tissues such as the lung, blood vessels and skin, microfibrils form the periphery of the elastic fiber, acting as a scaffold for the deposition of elastin (PubMed:27026396). In addition, microfibrils can occur as elastin-independent networks in tissues such as the ciliary zonule, tendon, cornea and glomerulus where they provide tensile strength and have anchoring roles (PubMed:27026396). Fibrillin-1 also plays a key role in tissue homeostasis through specific interactions with growth factors, such as the bone morphogenetic proteins (BMPs), growth and differentiation factors (GDFs) and latent transforming growth factor-beta-binding proteins (LTBPs), cell-surface integrins and other extracellular matrix protein and proteoglycan components (PubMed:27026396). Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively (By similarity). Negatively regulates osteoclastogenesis by binding and sequestering an osteoclast differentiation and activation factor TNFSF11 (PubMed:24039232). This leads to disruption of TNFSF11-induced Ca(2+) signaling and impairment of TNFSF11-mediated nuclear translocation and activation of transcription factor NFATC1 which regulates genes important for osteoclast differentiation and function (PubMed:24039232). Mediates cell adhesion via its binding to cell surface receptors integrins ITGAV:ITGB3 and ITGA5:ITGB1 (PubMed:12807887, PubMed:17158881). Binds heparin and this interaction has an important role in the assembly of microfibrils (PubMed:11461921)

Protein Sequence

10 MRRGRLLEIA 20 LGFTVLLASY 30 TSHGADANLE 40 AGNVKETRAS 50 RAKRRGGGGH 60 DALKGPNVCG 70 SRYNAYCCPG 80 WKTLPGGNQC 90 IVPICRHSCG 100 DGFCSRPNMC 110 TCPSGQIAPS 120 CGSRSIQHCN 130 IRCMNGGSCS 140 DDHCLCQKGY 150 IGTHCGQPVC 160 ESGCLNGGRC 170 VAPNRCACTY 180 GFTGPQCERD 190 YRTGPCFTVI 200 SNQMCQGQLS 210 GIVCTKTLCC 220 ATVGRAWGHP 230 CEMCPAQPHP 240 CRRGFIPNIR 250 TGACQDVDEC 260 QAIPGLCQGG 270 NCINTVGSFE 280 CKCPAGHKLN 290 EVSQKCEDID 300 ECSTIPGICE 310 GGECTNTVSS 320 YFCKCPPGFY 330 TSPDGTRCID 340 VRPGYCYTAL 350 TNGRCSNQLP 360 QSITKMQCCC 370 DAGRCWSPGV 380 TVAPEMCPIR 390 ATEDFNKLCS 400 VPMVIPGRPE 410 YPPPPLGPIP 420 PVLPVPPGFP 430 PGPQIPVPRP 440 PVEYLYPSRE 450 PPRVLPVNVT 460 DYCQLVRYLC 470 QNGRCIPTPG 480 SYRCECNKGF 490 QLDLRGECID 500 VDECEKNPCA 510 GGECINNQGS 520 YTCQCRAGYQ 530 STLTRTECRD 540 IDECLQNGRI 550 CNNGRCINTD 560 GSFHCVCNAG 570 FHVTRDGKNC 580 EDMDECSIRN 590 MCLNGMCINE 600 DGSFKCICKP 610 GFQLASDGRY 620 CKDINECETP 630 GICMNGRCVN 640 TDGSYRCECF 650 PGLAVGLDGR 660 VCVDTHMRST 670 CYGGYKRGQC 680 IKPLFGAVTK 690 SECCCASTEY 700 AFGEPCQPCP 710 AQNSAEYQAL 720 CSSGPGMTSA 730 GSDINECALD 740 PDICPNGICE 750 NLRGTYKCIC 760 NSGYEVDSTG 770 KNCVDINECV 780 LNSLLCDNGQ 790 CRNTPGSFVC 800 TCPKGFIYKP 810 DLKTCEDIDE 820 CESSPCINGV 830 CKNSPGSFIC 840 ECSSESTLDP 850 TKTICIETIK 860 GTCWQTVIDG 870 RCEININGAT 880 LKSQCCSSLG 890 AAWGSPCTLC 900 QVDPICGKGY 910 SRIKGTQCED 920 IDECEVFPGV 930 CKNGLCVNTR 940 GSFKCQCPSG 950 MTLDATGRIC 960 LDIRLETCFL 970 RYEDEECTLP 980 IAGRHRMDAC 990 CCSVGAAWGT 1000 EECEECPMRN 1010 TPEYEELCPR 1020 GPGFATKEIT 1030 NGKPFFKDIN 1040 ECKMIPSLCT 1050 HGKCRNTIGS 1060 FKCRCDSGFA 1070 LDSEERNCTD 1080 IDECRISPDL 1090 CGRGQCVNTP 1100 GDFECKCDEG 1110 YESGFMMMKN 1120 CMDIDECQRD 1130 PLLCRGGVCH 1140 NTEGSYRCEC 1150 PPGHQLSPNI 1160 SACIDINECE 1170 LSAHLCPNGR 1180 CVNLIGKYQC 1190 ACNPGYHSTP 1200 DRLFCVDIDE 1210 CSIMNGGCET 1220 FCTNSEGSYE 1230 CSCQPGFALM 1240 PDQRSCTDID 1250 ECEDNPNICD 1260 GGQCTNIPGE 1270 YRCLCYDGFM 1280 ASEDMKTCVD 1290 VNECDLNPNI 1300 CLSGTCENTK 1310 GSFICHCDMG 1320 YSGKKGKTGC 1330 TDINECEIGA 1340 HNCGKHAVCT 1350 NTAGSFKCSC 1360 SPGWIGDGIK 1370 CTDLDECSNG 1380 THMCSQHADC 1390 KNTMGSYRCL 1400 CKEGYTGDGF 1410 TCTDLDECSE 1420 NLNLCGNGQC 1430 LNAPGGYRCE 1440 CDMGFVPSAD 1450 GKACEDIDEC 1460 SLPNICVFGT 1470 CHNLPGLFRC 1480 ECEIGYELDR 1490 SGGNCTDVNE 1500 CLDPTTCISG 1510 NCVNTPGSYI 1520 CDCPPDFELN 1530 PTRVGCVDTR 1540 SGNCYLDIRP 1550 RGDNGDTACS 1560 NEIGVGVSKA 1570 SCCCSLGKAW 1580 GTPCEMCPAV 1590 NTSEYKILCP 1600 GGEGFRPNPI 1610 TVILEDIDEC 1620 QELPGLCQGG 1630 KCINTFGSFQ 1640 CRCPTGYYLN 1650 EDTRVCDDVN 1660 ECETPGICGP 1670 GTCYNTVGNY 1680 TCICPPDYMQ 1690 VNGGNNCMDM 1700 RRSLCYRNYY 1710 ADNQTCDGEL 1720 LFNMTKKMCC 1730 CSYNIGRAWN 1740 KPCEQCPIPS 1750 TDEFATLCGS 1760 QRPGFVIDIY 1770 TGLPVDIDEC 1780 REIPGVCENG 1790 VCINMVGSFR 1800 CECPVGFFYN 1810 DKLLVCEDID 1820 ECQNGPVCQR 1830 NAECINTAGS 1840 YRCDCKPGYR 1850 FTSTGQCNDR 1860 NECQEIPNIC 1870 SHGQCIDTVG 1880 SFYCLCHTGF 1890 KTNDDQTMCL 1900 DINECERDAC 1910 GNGTCRNTIG 1920 SFNCRCNHGF 1930 ILSHNNDCID 1940 VDECASGNGN 1950 LCRNGQCINT 1960 VGSFQCQCNE 1970 GYEVAPDGRT 1980 CVDINECLLE 1990 PRKCAPGTCQ 2000 NLDGSYRCIC 2010 PPGYSLQNEK 2020 CEDIDECVEE 2030 PEICALGTCS 2040 NTEGSFKCLC 2050 PEGFSLSSSG 2060 RRCQDLRMSY 2070 CYAKFEGGKC 2080 SSPKSRNHSK 2090 QECCCALKGE 2100 GWGDPCELCP 2110 TEPDEAFRQI 2120 CPYGSGIIVG 2130 PDDSAVDMDE 2140 CKEPDVCKHG 2150 QCINTDGSYR 2160 CECPFGYILA 2170 GNECVDTDEC 2180 SVGNPCGNGT 2190 CKNVIGGFEC 2200 TCEEGFEPGP 2210 MMTCEDINEC 2220 AQNPLLCAFR 2230 CVNTYGSYEC 2240 KCPVGYVLRE 2250 DRRMCKDEDE 2260 CEEGKHDCTE 2270 KQMECKNLIG 2280 TYMCICGPGY 2290 QRRPDGEGCV 2300 DENECQTKPG 2310 ICENGRCLNT 2320 RGSYTCECND 2330 GFTASPNQDE 2340 CLDNREGYCF 2350 TEVLQNMCQI 2360 GSSNRNPVTK 2370 SECCCDGGRG 2380 WGPHCEICPF 2390 QGTVAFKKLC 2400 PHGRGFMTNG 2410 ADIDECKVIH 2420 DVCRNGECVN 2430 DRGSYHCICK 2440 TGYTPDITGT 2450 SCVDLNECNQ 2460 APKPCNFICK 2470 NTEGSYQCSC 2480 PKGYILQEDG 2490 RSCKDLDECA 2500 TKQHNCQFLC 2510 VNTIGGFTCK 2520 CPPGFTQHHT 2530 SCIDNNECTS 2540 DINLCGSKGI 2550 CQNTPGSFTC 2560 ECQRGFSLDQ 2570 TGSSCEDVDE 2580 CEGNHRCQHG 2590 CQNIIGGYRC 2600 SCPQGYLQHY 2610 QWNQCVDENE 2620 CLSAHICGGA 2630 SCHNTLGSYK 2640 CMCPAGFQYE 2650 QFSGGCQDIN 2660 ECGSAQAPCS 2670 YGCSNTEGGY 2680 LCGCPPGYFR 2690 IGQGHCVSGM 2700 GMGRGNPEPP 2710 VSGEMDDNSL 2720 SPEACYECKI 2730 NGYPKRGRKR 2740 RSTNETDASN 2750 IEDQSETEAN 2760 VSLASWDVEK 2770 TAIFAFNISH 2780 VSNKVRILEL 2790 LPALTTLTNH 2800 NRYLIESGNE 2810 DGFFKINQKE 2820 GISYLHFTKK 2830 KPVAGTYSLQ 2840 ISSTPLYKKK 2850 ELNQLEDKYD 2860 KDYLSGELGD 2870 NLKMKIQVLL H

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005604 basement membrane
Cellular Component GO:0005788 endoplasmic reticulum lumen
Cellular Component GO:0031012 extracellular matrix
Cellular Component GO:0005576 extracellular region
Cellular Component GO:0005615 extracellular space
Cellular Component GO:0001527 microfibril
Molecular Function GO:0005509 calcium ion binding
Molecular Function GO:0030023 extracellular matrix constituent conferring elasticity
Molecular Function GO:0005201 extracellular matrix structural constituent
Molecular Function GO:0008201 heparin binding
Molecular Function GO:0005179 hormone activity
Molecular Function GO:0042802 identical protein binding
Molecular Function GO:0005178 integrin binding
Molecular Function GO:0044877 protein-containing complex binding
Biological Process GO:0043010 camera-type eye development
Biological Process GO:0033627 cell adhesion mediated by integrin
Biological Process GO:1990314 cellular response to insulin-like growth factor stimulus
Biological Process GO:0071560 cellular response to transforming growth factor beta stimulus
Biological Process GO:0048048 embryonic eye morphogenesis
Biological Process GO:0042593 glucose homeostasis
Biological Process GO:0006006 glucose metabolic process
Biological Process GO:0007507 heart development
Biological Process GO:0001656 metanephros development
Biological Process GO:0030514 negative regulation of BMP signaling pathway
Biological Process GO:2001205 negative regulation of osteoclast development
Biological Process GO:0045671 negative regulation of osteoclast differentiation
Biological Process GO:0030512 negative regulation of transforming growth factor beta receptor signaling pathway
Biological Process GO:0048050 post-embryonic eye morphogenesis
Biological Process GO:0001501 skeletal system development

Reference

[1] Hong H, Chen X, Wang H, Gu X, Yuan Y et al.. Global profiling of protein lysine lactylation and potential target modified protein analysis in hepatocellular carcinoma.. Proteomics 23(9):e2200432. 2023 May. PMID: 36625413.

[2] Lin Y, Chen M, Wang D, Yu Y, Chen R et al.. Multi-Proteomic Analysis Reveals the Effect of Protein Lactylation on Matrix and Cholesterol Metabolism in Tendinopathy.. J Proteome Res 22(6):1712-1722. 2023 Jun 2. PMID: 37159428.

[3] He J, Lai T, Zhou Z, Yang H, Lei Z et al.. Multiomics profiling reveals the involvement of protein lactylation in nonhomologous end joining pathway conferring radioresistance in lung adenocarcinoma cell.. Sci Rep 15(1):24651. 2025 Jul 9. PMID: 40634431.