Protein: Fibrillin-1 [Cleaved into: Asprosin] (Q61554)
Organism: Mus musculus (Mouse)
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. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. 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. 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. 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 (By similarity). Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Negatively regulates osteoclastogenesis by binding and sequestering an osteoclast differentiation and activation factor TNFSF11. 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. Mediates cell adhesion via its binding to cell surface receptors integrins ITGAV:ITGB3 and ITGA5:ITGB1. Binds heparin and this interaction plays an important role in the assembly of microfibrils (By similarity).
Subcellular location: Secreted {ECO:0000250|UniProtKB:P35555}. Note=Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin. {ECO:0000250|UniProtKB:P35555}.; SUBCELLULAR LOCATION: [Fibrillin-1]: Secreted, extracellular space, extracellular matrix {ECO:0000269|PubMed:24039232}.; SUBCELLULAR LOCATION: [Asprosin]: Secreted {ECO:0000250|UniProtKB:P35555}. Note=Secreted by white adipose tissue and circulates in the plasma. {ECO:0000250|UniProtKB:P35555}.
Interaction partners:
- Elastin
- Latent-transforming growth factor beta-binding protein 1
- Microfibrillar-associated protein 2
- A disintegrin and metalloproteinase with thrombospondin motifs 10
- Lumican
- Microfibrillar-associated protein 5
- Fibulin-2
- Collagen alpha-1
- Collagen alpha-2
- Fibronectin
Sequence:
MRRGGLLEVALAFALLLESYTSHGADANLEAGSLKETRANRAKRRGGGGHDALKGPNVCGSRYNAYCCPGWKTLPGGNQCIVPICRHSCGDGFCSRPNMCTCPSGQISPSCGSRSIQHCSIRCMNGGSCSDDHCLCQKGYIGTHCGQPVCESGCLNGGRCVAPNRCACTYGFTGPQCERDYRTGPCFTVVSNQMCQGQLSGIVCTKTLCCATVGRAWGHPCEMCPAQPHPCRRGFIPNIRTGACQDVDECQAIPGMCQGGNCINTVGSFECKCPAGHKFNEVSQKCEDIDECSTIPGVCDGGECTNTVSSYFCKCPPGFYTSPDGTRCVDVRPGYCYTALANGRCSNQLPQSITKMQCCCDLGRCWSPGVTVAPEMCPIRSTEDFNKLCSVPLVIPGRPEYPPPPIGPLPPVQPVPPGYPPGPVIPAPRPPPEYPYPSPSREPPRVLPFNVTDYCQLVRYLCQNGRCIPTPGSYRCECNKGFQLDIRGECIDVDECEKNPCTGGECINNQGSYTCHCRAGYQSTLTRTECRDIDECLQNGRICNNGRCINTDGSFHCVCNAGFHVTRDGKNCEDMDECSIRNMCLNGMCINEDGSFKCICKPGFQLASDGRYCKDINECETPGICMNGRCVNTDGSYRCECFPGLAVGLDGRVCVDTHMRSTCYGGYRRGQCVKPLFGAVTKSECCCASTEYAFGEPCQPCPAQNSAEYQALCSSGPGMTSAGTDINECALDPDICPNGICENLRGTYKCICNSGYEVDITGKNCVDINECVLNSLLCDNGQCRNTPGSFVCTCPKGFVYKPDLKTCEDIDECESSPCINGVCKNSPGSFICECSPESTLDPTKTICIETIKGTCWQTVIDGRCEININGATLKSECCSSLGAAWGSPCTICQLDPICGKGFSRIKGTQCEDINECEVFPGVCKNGLCVNSRGSFKCECPNGMTLDATGRICLDIRLETCFLKYDDEECTLPIAGRHRMDACCCSVGAAWGTEECEECPLRNSREYEELCPRGPGFATKDITNGKPFFKDINECKMIPSLCTHGKCRNTIGSFKCRCDSGFALDSEERNCTDIDECRISPDLCGRGQCVNTPGDFECKCDEGYESGFMMMKNCMDIDECQRDPLLCRGGICHNTEGSYRCECPPGHQLSPNISACIDINECELSANLCPHGRCVNLIGKYQCACNPGYHPTHDRLFCVDIDECSIMNGGCETFCTNSDGSYECSCQPGFALMPDQRSCTDIDECEDNPNICDGGQCTNIPGEYRCLCYDGFMASEDMKTCVDVNECDLNPNICLSGTCENTKGSFICHCDMGYSGKKGKTGCTDINECEIGAHNCGRHAVCTNTAGSFKCSCSPGWIGDGIKCTDLDECSNGTHMCSQHADCKNTMGSYRCLCKDGYTGDGFTCTDLDECSENLNLCGNGQCLNAPGGYRCECDMGFVPSADGKACEDIDECSLPNICVFGTCHNLPGLFRCECEIGYELDRSGGNCTDVNECLDPTTCISGNCVNTPGSYTCDCPPDFELNPTRVGCVDTRSGNCYLDIRPRGDNGDTACSNEIGVGVSKASCCCSLGKAWGTPCELCPSVNTSEYKILCPGGEGFRPNPITVILEDIDECQELPGLCQGGKCINTFGSFQCRCPTGYYLNEDTRVCDDVNECETPGICGPGTCYNTVGNYTCICPPDYMQVNGGNNCMDMRRSLCYRNYYADNQTCDGELLFNMTKKMCCCSYNIGRAWNKPCEQCPIPSTDEFATLCGSQRPGFVIDIYTGLPVDIDECREIPGVCENGVCINMVGSFRCECPVGFFYNDKLLVCEDIDECQNGPVCQRNAECINTAGSYRCDCKPGYRLTSTGQCNDRNECQEIPNICSHGQCIDTVGSFYCLCHTGFKTNVDQTMCLDINECERDACGNGTCRNTIGSFNCRCNHGFILSHNNDCIDVDECATGNGNLCRNGQCVNTVGSFQCRCNEGYEVAPDGRTCVDINECVLDPGKCAPGTCQNLDGSYRCICPPGYSLQNDKCEDIDECVEEPEICALGTCSNTEGSFKCLCPEGFSLSSTGRRCQDLRMSYCYAKFEGGKCSSPKSRNHSKQECCCALKGEGWGDPCELCPTEPDEAFRQICPFGSGIIVGPDDSAVDMDECKEPDVCRHGQCINTDGSYRCECPFGYILEGNECVDTDECSVGNPCGNGTCKNVIGGFECTCEEGFEPGPMMTCEDINECAQNPLLCAFRCVNTYGSYECKCPVGYVLREDRRMCKDEDECAEGKHDCTEKQMECKNLIGTYMCICGPGYQRRPDGEGCIDENECQTKPGICENGRCLNTLGSYTCECNDGFTASPTQDECLDNREGYCFSEVLQNMCQIGSSNRNPVTKSECCCDGGRGWGPHCEICPFEGTVAYKKLCPHGRGFMTNGADIDECKVIHDVCRNGECVNDRGSYHCICKTGYTPDITGTACVDLNECNQAPKPCNFICKNTEGSYQCSCPKGYILQEDGRSCKDLDECATKQHNCQFLCVNTIGGFTCKCPPGFTQHHTACIDNNECTSDINLCGSKGVCQNTPGSFTCECQRGFSLDQSGASCEDVDECEGNHRCQHGCQNIIGGYRCSCPQGYLQHYQWNQCVDENECLSAHVCGGASCHNTLGSYKCMCPTGFQYEQFSGGCQDINECGSSQAPCSYGCSNTEGGYLCGCPPGYFRIGQGHCVSGMGMGRGGPEPPASSEMDDNSLSPEACYECKINGYPKRGRKRRSTNETDASDIQDGSEMEANVSLASWDVEKPASFAFNISHVNNKVRILELLPALTTLMNHNRYLIESGNEDGFFKINQKEGVSYLHFTKKKPVAGTYSLQISSTPLYKKKELNQLEDRYDKDYLSGELGDNLKMKIQILLH