UniProt Accession

 FBN1_HUMAN; P35555; B2RUU0; D2JYH6; Q15972; Q75N87 

Genbank Protein ID

 L13923; AB177803; GU143398; AC022467; AC084757; AC084758; CH471082; BC146854; L19896; X63556; X62008; S54426; S54425 

Genbank Nucleotide ID

 AAB02036.1; BAD16739.1; ACZ58372.1; EAW77354.1; AAI46855.1; CAA45118.1; AAB25244.1; AAB25244.1 

Protein Name

 Fibrillin-1 

Protein Synonyms/Alias

  

Gene Name

 FBN1 

Gene Synonyms/Alias

 FBN 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
599	GSFKCICKPGFQLAS	ubiquitination	[1]
1096	TPGDFECKCDEGYES	ubiquitination	[1]
2246	REDRRMCKDEDECEE	ubiquitination	[1]
2255	EDECEEGKHDCTEKQ	ubiquitination	[1]
2261	GKHDCTEKQMECKNL	ubiquitination	[1]

Reference

 [1] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661] 

Functional Description

 Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1- containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. 

Sequence Annotation

 DOMAIN 81 112 EGF-like 1.
 DOMAIN 115 146 EGF-like 2.
 DOMAIN 147 178 EGF-like 3.
 DOMAIN 184 236 TB 1.
 DOMAIN 246 287 EGF-like 4; calcium-binding.
 DOMAIN 288 329 EGF-like 5; calcium-binding.
 DOMAIN 334 389 TB 2.
 DOMAIN 449 489 EGF-like 6.
 DOMAIN 490 529 EGF-like 7; calcium-binding.
 DOMAIN 530 571 EGF-like 8; calcium-binding.
 DOMAIN 572 612 EGF-like 9; calcium-binding.
 DOMAIN 613 653 EGF-like 10; calcium-binding.
 DOMAIN 659 711 TB 3.
 DOMAIN 723 764 EGF-like 11; calcium-binding.
 DOMAIN 765 806 EGF-like 12; calcium-binding.
 DOMAIN 807 846 EGF-like 13; calcium-binding.
 DOMAIN 851 902 TB 4.
 DOMAIN 910 951 EGF-like 14; calcium-binding.
 DOMAIN 956 1008 TB 5.
 DOMAIN 1028 1069 EGF-like 15; calcium-binding.
 DOMAIN 1070 1112 EGF-like 16; calcium-binding.
 DOMAIN 1113 1154 EGF-like 17; calcium-binding.
 DOMAIN 1155 1196 EGF-like 18; calcium-binding.
 DOMAIN 1197 1237 EGF-like 19; calcium-binding.
 DOMAIN 1238 1279 EGF-like 20; calcium-binding.
 DOMAIN 1280 1321 EGF-like 21; calcium-binding.
 DOMAIN 1322 1362 EGF-like 22; calcium-binding.
 DOMAIN 1363 1403 EGF-like 23; calcium-binding.
 DOMAIN 1404 1445 EGF-like 24; calcium-binding.
 DOMAIN 1446 1486 EGF-like 25; calcium-binding.
 DOMAIN 1487 1527 EGF-like 26; calcium-binding.
 DOMAIN 1532 1589 TB 6.
 DOMAIN 1606 1647 EGF-like 27; calcium-binding.
 DOMAIN 1648 1688 EGF-like 28; calcium-binding.
 DOMAIN 1693 1748 TB 7.
 DOMAIN 1766 1807 EGF-like 29; calcium-binding.
 DOMAIN 1808 1848 EGF-like 30; calcium-binding.
 DOMAIN 1849 1890 EGF-like 31; calcium-binding.
 DOMAIN 1891 1929 EGF-like 32; calcium-binding.
 DOMAIN 1930 1972 EGF-like 33; calcium-binding.
 DOMAIN 1973 2012 EGF-like 34; calcium-binding.
 DOMAIN 2013 2054 EGF-like 35; calcium-binding.
 DOMAIN 2059 2111 TB 8.
 DOMAIN 2127 2165 EGF-like 36; calcium-binding.
 DOMAIN 2166 2205 EGF-like 37; calcium-binding.
 DOMAIN 2206 2246 EGF-like 38; calcium-binding.
 DOMAIN 2247 2290 EGF-like 39; calcium-binding.
 DOMAIN 2291 2332 EGF-like 40; calcium-binding.
 DOMAIN 2337 2390 TB 9.
 DOMAIN 2402 2443 EGF-like 41; calcium-binding.
 DOMAIN 2444 2484 EGF-like 42; calcium-binding.
 DOMAIN 2485 2523 EGF-like 43; calcium-binding.
 DOMAIN 2524 2566 EGF-like 44; calcium-binding.
 DOMAIN 2567 2606 EGF-like 45; calcium-binding.
 DOMAIN 2607 2647 EGF-like 46; calcium-binding.
 DOMAIN 2648 2687 EGF-like 47; calcium-binding.
 MOD_RES 1103 1103 Phosphoserine (By similarity).
 MOD_RES 2071 2071 Phosphoserine (By similarity).
 CARBOHYD 448 448 N-linked (GlcNAc...).
 CARBOHYD 1067 1067 N-linked (GlcNAc...).
 CARBOHYD 1149 1149 N-linked (GlcNAc...) (Potential).
 CARBOHYD 1369 1369 N-linked (GlcNAc...) (Potential).
 CARBOHYD 1484 1484 N-linked (GlcNAc...).
 CARBOHYD 1581 1581 N-linked (GlcNAc...).
 CARBOHYD 1669 1669 N-linked (GlcNAc...) (Potential).
 CARBOHYD 1703 1703 N-linked (GlcNAc...) (Potential).
 CARBOHYD 1713 1713 N-linked (GlcNAc...) (Potential).
 CARBOHYD 1902 1902 N-linked (GlcNAc...) (Potential).
 CARBOHYD 2077 2077 N-linked (GlcNAc...) (Potential).
 CARBOHYD 2178 2178 N-linked (GlcNAc...) (Potential).
 CARBOHYD 2734 2734 N-linked (GlcNAc...) (Potential).
 CARBOHYD 2750 2750 N-linked (GlcNAc...) (Potential).
 CARBOHYD 2767 2767 N-linked (GlcNAc...) (Potential).
 DISULFID 85 94 By similarity.
 DISULFID 89 100 By similarity.
 DISULFID 102 111 By similarity.
 DISULFID 119 129 By similarity.
 DISULFID 123 134 By similarity.
 DISULFID 136 145 By similarity.
 DISULFID 150 160 By similarity.
 DISULFID 154 166 By similarity.
 DISULFID 168 177 By similarity.
 DISULFID 250 262 By similarity.
 DISULFID 257 271 By similarity.
 DISULFID 273 286 By similarity.
 DISULFID 292 304 By similarity.
 DISULFID 299 313 By similarity.
 DISULFID 315 328 By similarity.
 DISULFID 453 465 By similarity.
 DISULFID 460 474 By similarity.
 DISULFID 476 488 By similarity.
 DISULFID 494 504 By similarity.
 DISULFID 499 513 By similarity.
 DISULFID 515 528 By similarity.
 DISULFID 534 546 By similarity.
 DISULFID 541 555 By similarity.
 DISULFID 557 570 By similarity.
 DISULFID 576 587 By similarity.
 DISULFID 582 596 By similarity.
 DISULFID 598 611 By similarity.
 DISULFID 617 628 By similarity.
 DISULFID 623 637 By similarity.
 DISULFID 639 652 By similarity.
 DISULFID 727 739 By similarity.
 DISULFID 734 748 By similarity.
 DISULFID 750 763 By similarity.
 DISULFID 769 781 By similarity.
 DISULFID 776 790 By similarity.
 DISULFID 792 805 By similarity.
 DISULFID 811 821
 DISULFID 816 830
 DISULFID 832 845
 DISULFID 853 875
 DISULFID 862 887
 DISULFID 876 890
 DISULFID 896 908
 DISULFID 914 926
 DISULFID 921 935
 DISULFID 937 950
 DISULFID 1032 1044 By similarity.
 DISULFID 1039 1053 By similarity.
 DISULFID 1055 1068 By similarity.
 DISULFID 1074 1086 By similarity.
 DISULFID 1081 1095 By similarity.
 DISULFID 1097 1111 By similarity.
 DISULFID 1117 1129 By similarity.
 DISULFID 1124 1138 By similarity.
 DISULFID 1140 1153 By similarity.
 DISULFID 1159 1171 By similarity.
 DISULFID 1166 1180 By similarity.
 DISULFID 1182 1195 By similarity.
 DISULFID 1201 1212 By similarity.
 DISULFID 1208 1221 By similarity.
 DISULFID 1223 1236 By similarity.
 DISULFID 1242 1254 By similarity.
 DISULFID 1249 1263 By similarity.
 DISULFID 1265 1278 By similarity.
 DISULFID 1284 1296 By similarity.
 DISULFID 1291 1305 By similarity.
 DISULFID 1307 1320 By similarity.
 DISULFID 1326 1339 By similarity.
 DISULFID 1333 1348 By similarity.
 DISULFID 1350 1361 By similarity.
 DISULFID 1367 1380 By similarity.
 DISULFID 1374 1389 By similarity.
 DISULFID 1391 1402 By similarity.
 DISULFID 1408 1420 By similarity.
 DISULFID 1415 1429 By similarity.
 DISULFID 1431 1444 By similarity.
 DISULFID 1450 1461 By similarity.
 DISULFID 1456 1470 By similarity.
 DISULFID 1472 1485 By similarity.
 DISULFID 1491 1502
 DISULFID 1497 1511
 DISULFID 1513 1526
 DISULFID 1534 1562
 DISULFID 1549 1574
 DISULFID 1563 1577
 DISULFID 1564 1589
 DISULFID 1610 1622
 DISULFID 1617 1631
 DISULFID 1633 1646
 DISULFID 1652 1663 By similarity.
 DISULFID 1658 1672 By similarity.
 DISULFID 1674 1687 By similarity.
 DISULFID 1770 1782 By similarity.
 DISULFID 1777 1791 By similarity.
 DISULFID 1793 1806 By similarity.
 DISULFID 1812 1824 By similarity.
 DISULFID 1818 1833 By similarity.
 DISULFID 1835 1847 By similarity.
 DISULFID 1853 1865 By similarity.
 DISULFID 1860 1874 By similarity.
 DISULFID 1876 1889 By similarity.
 DISULFID 1895 1905 By similarity.
 DISULFID 1900 1914 By similarity.
 DISULFID 1916 1928 By similarity.
 DISULFID 1934 1947 By similarity.
 DISULFID 1942 1956 By similarity.
 DISULFID 1958 1971 By similarity.
 DISULFID 1977 1989 By similarity.
 DISULFID 1984 1998 By similarity.
 DISULFID 2000 2011 By similarity.
 DISULFID 2017 2029 By similarity.
 DISULFID 2024 2038 By similarity.
 DISULFID 2040 2053 By similarity.
 DISULFID 2061 2083
 DISULFID 2070 2096
 DISULFID 2084 2099
 DISULFID 2085 2111
 DISULFID 2131 2142 By similarity.
 DISULFID 2137 2151 By similarity.
 DISULFID 2153 2164 By similarity.
 DISULFID 2170 2181 By similarity.
 DISULFID 2176 2190 By similarity.
 DISULFID 2192 2204 By similarity.
 DISULFID 2210 2221 By similarity.
 DISULFID 2217 2230 By similarity.
 DISULFID 2232 2245 By similarity.
 DISULFID 2251 2265 By similarity.
 DISULFID 2258 2274 By similarity.
 DISULFID 2276 2289 By similarity.
 DISULFID 2295 2307 By similarity.
 DISULFID 2302 2316 By similarity.
 DISULFID 2318 2331 By similarity.
 DISULFID 2406 2418 By similarity.
 DISULFID 2413 2427 By similarity.
 DISULFID 2429 2442 By similarity.
 DISULFID 2448 2459 By similarity.
 DISULFID 2455 2468 By similarity.
 DISULFID 2470 2483 By similarity.
 DISULFID 2489 2500 By similarity.
 DISULFID 2496 2509 By similarity.
 DISULFID 2511 2522 By similarity.
 DISULFID 2528 2541 By similarity.
 DISULFID 2535 2550 By similarity.
 DISULFID 2552 2565 By similarity.
 DISULFID 2571 2581 By similarity.
 DISULFID 2577 2590 By similarity.
 DISULFID 2592 2605 By similarity.
 DISULFID 2611 2622 By similarity.
 DISULFID 2617 2631 By similarity.
 DISULFID 2633 2646 By similarity.
 DISULFID 2652 2663 By similarity.
 DISULFID 2659 2672 By similarity.
 DISULFID 2674 2686 By similarity.  

Keyword

 3D-structure; Aortic aneurysm; Calcium; Complete proteome; Craniosynostosis; Disease mutation; Disulfide bond; Dwarfism; EGF-like domain; Extracellular matrix; Glycoprotein; Phosphoprotein; Polymorphism; Reference proteome; Repeat; Secreted; Signal. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 2871 AA 

Protein Sequence

Gene Ontology

 GO:0005604; C:basement membrane; IDA:UniProtKB.
 GO:0005615; C:extracellular space; IDA:UniProtKB.
 GO:0001527; C:microfibril; IDA:UniProtKB.
 GO:0005509; F:calcium ion binding; IDA:UniProtKB.
 GO:0005201; F:extracellular matrix structural constituent; IDA:UniProtKB.
 GO:0030198; P:extracellular matrix organization; TAS:Reactome.
 GO:0007507; P:heart development; IMP:UniProtKB.
 GO:0035582; P:sequestering of BMP in extracellular matrix; ISS:BHF-UCL.
 GO:0035583; P:sequestering of TGFbeta in extracellular matrix; ISS:BHF-UCL.
 GO:0001501; P:skeletal system development; IMP:UniProtKB. 

Interpro

 IPR026823; cEGF.
 IPR000742; EG-like_dom.
 IPR001881; EGF-like_Ca-bd.
 IPR013032; EGF-like_CS.
 IPR000152; EGF-type_Asp/Asn_hydroxyl_site.
 IPR018097; EGF_Ca-bd_CS.
 IPR011398; FBN.
 IPR009030; Growth_fac_rcpt_N_dom.
 IPR017878; TB_dom. 

Pfam

 PF12662; cEGF
 PF07645; EGF_CA
 PF00683; TB 

SMART

 SM00181; EGF
 SM00179; EGF_CA 

PROSITE

 PS00010; ASX_HYDROXYL
 PS00022; EGF_1
 PS01186; EGF_2
 PS50026; EGF_3
 PS01187; EGF_CA
 PS51364; TB 

PRINTS