{"product_id":"fbn1-antibody-fitc-conjugated-cat-csb-pa008456lc01hu","title":"FBN1 Antibody, FITC conjugated - Cat. #: CSB-PA008456LC01HU","description":"\u003cp\u003e\u003cb\u003eSize \u003c\/b\u003e:50ug\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e:\u003c\/p\u003e\u003cp\u003e\u003cb\u003eAliases\u003c\/b\u003e:350 kDa glycoprotein component extracellular microfibril antibody; ACMICD antibody; FBN 1 antibody; FBN antibody; FBN1 antibody; FBN1_HUMAN antibody; Fibrillin 15 antibody; Fibrillin-1 antibody; Fibrillin1 antibody; Fibrillin15 antibody; GPHYSD2 antibody; Marfan syndrome antibody; MASS antibody; MFS 1 antibody; MFS1 antibody; OCTD antibody; SGS antibody; SSKS antibody; Weill Marchesani syndrome  antibody; WMS antibody; WMS2 antibody\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProduct Type\u003c\/b\u003e:Polyclonal Antibody\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen Species\u003c\/b\u003e:Homo sapiens (Human)\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniProt ID\u003c\/b\u003e:P35555\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e:Recombinant Human Fibrillin-1 protein (783-850AA)\u003c\/p\u003e\u003cp\u003e\u003cb\u003eRaised in\u003c\/b\u003e:Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecies Reactivity\u003c\/b\u003e:Human\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTested Applications\u003c\/b\u003e:\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e:Fibrillin-1: 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:1860873, PubMed:15062093). 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 (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. This leads to disruption of TNFSF11-induced Ca2+ 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).\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e:Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e:IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification Method\u003c\/b\u003e:\u0026gt;95%, Protein G purified\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e:FITC\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBuffer\u003c\/b\u003e:Preservative: 0.03% Proclin 300\u003cbr\u003eConstituents: 50% Glycerol, 0.01M PBS, pH 7.4\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e:Liquid\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStroage\u003c\/b\u003e:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTarget Names\u003c\/b\u003e:FBN1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eResearch Areas\u003c\/b\u003e:Signal transduction\u003c\/p\u003e","brand":"Gene Bio Systems","offers":[{"title":"Default Title","offer_id":40487082164324,"sku":"CSB-PA008456LC01HU","price":409.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/products\/no_image_default_image-jpeg_fa010423-5be9-498c-ada5-25c3ff6f8fcc.jpg?v=1660158870","url":"https:\/\/www.genebiosystems.com\/en-us\/products\/fbn1-antibody-fitc-conjugated-cat-csb-pa008456lc01hu","provider":"GeneBio ","version":"1.0","type":"link"}