Names & Taxonomy

Uniprot ID:
P35555
Entry Name:
FBN1_HUMAN
Status:
reviewed
Protein Names:
Fibrillin-1
Gene Names:
FBN1 FBN
Gene Names Primary:
FBN1
Organism:
Homo sapiens (Human)

Structure

Length:
2871
Sequence:
MRRGRLLEIALGFTVLLASYTSHGADANLEAGNVKETRASRAKRRGGGGHDALKGPNVCGSRYNAYCCPGWKTLPGGNQCIVPICRHSCGDGFCSRPNMCTCPSGQIAPSCGSRSIQHCNIRCMNGGSCSDDHCLCQKGYIGTHCGQPVCESGCLNGGRCVAPNRCACTYGFTGPQCERDYRTGPCFTVISNQMCQGQLSGIVCTKTLCCATVGRAWGHPCEMCPAQPHPCRRGFIPNIRTGACQDVDECQAIPGLCQGGNCINTVGSFECKCPAGHKLNEVSQKCEDIDECSTIPGICEGGECTNTVSSYFCKCPPGFYTSPDGTRCIDVRPGYCYTALTNGRCSNQLPQSITKMQCCCDAGRCWSPGVTVAPEMCPIRATEDFNKLCSVPMVIPGRPEYPPPPLGPIPPVLPVPPGFPPGPQIPVPRPPVEYLYPSREPPRVLPVNVTDYCQLVRYLCQNGRCIPTPGSCRCECNKGFQLDLRGECIDVDECEKNPCAGGECINNQGSYTCQCRAGYQSTLTRTECRDIDECLQNGRICNNGRCINTDGSFHCVCNAGFHVTRDGKNCEDMDECSIRNMCLNGMCINEDGSFKCICKPGFQLASDGRYCKDINECETPGICMNGRCVNTDGSYRCECFPGLAVGLDGRVCVDTHMRSTCYGGYKRGQCIKPLFGAVTKSECCCASTEYAFGEPCQPCPAQNSAEYQALCSSGPGMTSAGSDINECALDPDICPNGICENLRGTYKCICNSGYEVDSTGKNCVDINECVLNSLLCDNGQCRNTPGSFVCTCPKGFIYKPDLKTCEDIDECESSPCINGVCKNSPGSFICECSSESTLDPTKTICIETIKGTCWQTVIDGRCEININGATLKSQCCSSLGAAWGSPCTLCQVDPICGKGYSRIKGTQCEDIDECEVFPGVCKNGLCVNTRGSFKCQCPSGMTLDATGRICLDIRLETCFLRYEDEECTLPIAGRHRMDACCCSVGAAWGTEECEECPMRNTPEYEELCPRGPGFATKEITNGKPFFKDINECKMIPSLCTHGKCRNTIGSFKCRCDSGFALDSEERNCTDIDECRISPDLCGRGQCVNTPGDFECKCDEGYESGFMMMKNCMDIDECQRDPLLCRGGVCHNTEGSYRCECPPGHQLSPNISACIDINECELSAHLCPNGRCVNLIGKYQCACNPGYHSTPDRLFCVDIDECSIMNGGCETFCTNSEGSYECSCQPGFALMPDQRSCTDIDECEDNPNICDGGQCTNIPGEYRCLCYDGFMASEDMKTCVDVNECDLNPNICLSGTCENTKGSFICHCDMGYSGKKGKTGCTDINECEIGAHNCGKHAVCTNTAGSFKCSCSPGWIGDGIKCTDLDECSNGTHMCSQHADCKNTMGSYRCLCKEGYTGDGFTCTDLDECSENLNLCGNGQCLNAPGGYRCECDMGFVPSADGKACEDIDECSLPNICVFGTCHNLPGLFRCECEIGYELDRSGGNCTDVNECLDPTTCISGNCVNTPGSYICDCPPDFELNPTRVGCVDTRSGNCYLDIRPRGDNGDTACSNEIGVGVSKASCCCSLGKAWGTPCEMCPAVNTSEYKILCPGGEGFRPNPITVILEDIDECQELPGLCQGGKCINTFGSFQCRCPTGYYLNEDTRVCDDVNECETPGICGPGTCYNTVGNYTCICPPDYMQVNGGNNCMDMRRSLCYRNYYADNQTCDGELLFNMTKKMCCCSYNIGRAWNKPCEQCPIPSTDEFATLCGSQRPGFVIDIYTGLPVDIDECREIPGVCENGVCINMVGSFRCECPVGFFYNDKLLVCEDIDECQNGPVCQRNAECINTAGSYRCDCKPGYRFTSTGQCNDRNECQEIPNICSHGQCIDTVGSFYCLCHTGFKTNDDQTMCLDINECERDACGNGTCRNTIGSFNCRCNHGFILSHNNDCIDVDECASGNGNLCRNGQCINTVGSFQCQCNEGYEVAPDGRTCVDINECLLEPRKCAPGTCQNLDGSYRCICPPGYSLQNEKCEDIDECVEEPEICALGTCSNTEGSFKCLCPEGFSLSSSGRRCQDLRMSYCYAKFEGGKCSSPKSRNHSKQECCCALKGEGWGDPCELCPTEPDEAFRQICPYGSGIIVGPDDSAVDMDECKEPDVCKHGQCINTDGSYRCECPFGYILAGNECVDTDECSVGNPCGNGTCKNVIGGFECTCEEGFEPGPMMTCEDINECAQNPLLCAFRCVNTYGSYECKCPVGYVLREDRRMCKDEDECEEGKHDCTEKQMECKNLIGTYMCICGPGYQRRPDGEGCVDENECQTKPGICENGRCLNTRGSYTCECNDGFTASPNQDECLDNREGYCFTEVLQNMCQIGSSNRNPVTKSECCCDGGRGWGPHCEICPFQGTVAFKKLCPHGRGFMTNGADIDECKVIHDVCRNGECVNDRGSYHCICKTGYTPDITGTSCVDLNECNQAPKPCNFICKNTEGSYQCSCPKGYILQEDGRSCKDLDECATKQHNCQFLCVNTIGGFTCKCPPGFTQHHTSCIDNNECTSDINLCGSKGICQNTPGSFTCECQRGFSLDQTGSSCEDVDECEGNHRCQHGCQNIIGGYRCSCPQGYLQHYQWNQCVDENECLSAHICGGASCHNTLGSYKCMCPAGFQYEQFSGGCQDINECGSAQAPCSYGCSNTEGGYLCGCPPGYFRIGQGHCVSGMGMGRGNPEPPVSGEMDDNSLSPEACYECKINGYPKRGRKRRSTNETDASNIEDQSETEANVSLASWDVEKTAIFAFNISHVSNKVRILELLPALTTLTNHNRYLIESGNEDGFFKINQKEGISYLHFTKKKPVAGTYSLQISSTPLYKKKELNQLEDKYDKDYLSGELGDNLKMKIQVLLH
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Secreted, extracellular space, extracellular matrix

Function

Function:
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.
Gene Ontology Go:
basement membrane
extracellular exosome
extracellular matrix
extracellular region
extracellular space
microfibril
proteinaceous extracellular matrix
calcium ion binding
extracellular matrix constituent conferring elasticity
extracellular matrix structural constituent
integrin binding
protein complex binding
camera-type eye development
cellular response to insulin-like growth factor stimulus
cellular response to transforming growth factor beta stimulus
embryonic eye morphogenesis
extracellular matrix disassembly
extracellular matrix organization
heart development
metanephros development
post-embryonic eye morphogenesis
regulation of cellular response to growth factor stimulus
sequestering of BMP in extracellular matrix
sequestering of TGFbeta in extracellular matrix
skeletal system development
Gene Ontology Biological Process:
camera-type eye development
cellular response to insulin-like growth factor stimulus
cellular response to transforming growth factor beta stimulus
embryonic eye morphogenesis
extracellular matrix disassembly
extracellular matrix organization
heart development
metanephros development
post-embryonic eye morphogenesis
regulation of cellular response to growth factor stimulus
sequestering of BMP in extracellular matrix
sequestering of TGFbeta in extracellular matrix
skeletal system development
Gene Ontology Molecular Function:
calcium ion binding
extracellular matrix constituent conferring elasticity
extracellular matrix structural constituent
integrin binding
protein complex binding
Gene Ontology Cellular Component:
basement membrane
extracellular exosome
extracellular matrix
extracellular region
extracellular space
microfibril
proteinaceous extracellular matrix
Keywords:
3D-structure
Aortic aneurysm
Calcium
Complete proteome
Disease mutation
Disulfide bond
Dwarfism
EGF-like domain
Extracellular matrix
Glycoprotein
Phosphoprotein
Polymorphism
Reference proteome
Repeat
Secreted
Signal
Interacts With:
O95967; Q9UBX5; P35556; P02751; P28300; Q14766-2

Publication

PubMed ID:
8364578 15221638 16572171 15489334 7691719 1852207 1852206 8430317 1860873 15165854 19159218 21402694 24275569 9362480 8568869 8653794 8594563 9016526 9401003 10633129 12203987 2739055 26091039 15062093 19446531 1852208 1301946 1569206 8406497 8504310 8281141 7977366 8188302 8004112 8040326 8071963 7870075 8136837 7762551 7611299 7738200 8882780 8863159 9254848 9150726 9338581 9338588 8988160 9837823 9452085 10694921 10441597 10425041 11700157 12203992 11826022 12525539 14695540 15161917 16222657 16220557 20803651 20547088 20375004 21683322 21542060