{"product_id":"fbn1-antibody-biotin-conjugated-abccs55927","title":"FBN1 Antibody, Biotin conjugated","description":"\u003ch4\u003eSize\u003c\/h4\u003e\u003cp\u003e100ug\u003c\/p\u003e\u003ch4\u003eClone Number\u003c\/h4\u003e\u003cp\u003e\u003c\/p\u003e\u003ch4\u003eAliases\u003c\/h4\u003e\u003cp\u003e350 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\u003ch4\u003eImmunogen Species\u003c\/h4\u003e\u003cp\u003eHomo sapiens (Human)\u003c\/p\u003e\u003ch4\u003eUniProt ID\u003c\/h4\u003e\u003cp\u003eP35555\u003c\/p\u003e\u003ch4\u003eImmunogen\u003c\/h4\u003e\u003cp\u003eRecombinant Human Fibrillin-1 protein (783-850AA)\u003c\/p\u003e\u003ch4\u003eRaised in\u003c\/h4\u003e\u003cp\u003eRabbit\u003c\/p\u003e\u003ch4\u003eSpecies Reactivity\u003c\/h4\u003e\u003cp\u003eHuman\u003c\/p\u003e\u003ch4\u003eTested Applications\u003c\/h4\u003e\u003cp\u003eELISA\u003c\/p\u003e\u003ch4\u003eBackground\u003c\/h4\u003e\u003cp\u003eFibrillin-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\u003ch4\u003eClonality\u003c\/h4\u003e\u003cp\u003ePolyclonal\u003c\/p\u003e\u003ch4\u003eIsotype\u003c\/h4\u003e\u003cp\u003eIgG\u003c\/p\u003e\u003ch4\u003ePurification Method\u003c\/h4\u003e\u003cp\u003e\u0026gt;95%, Protein G purified\u003c\/p\u003e\u003ch4\u003eConjugate\u003c\/h4\u003e\u003cp\u003eBiotin\u003c\/p\u003e\u003ch4\u003eBuffer\u003c\/h4\u003e\u003cp\u003ePreservative: 0.03% Proclin 300\u003cbr\u003eConstituents: 50% Glycerol, 0.01M PBS, pH 7.4\u003c\/p\u003e\u003ch4\u003eForm\u003c\/h4\u003e\u003cp\u003eLiquid\u003c\/p\u003e\u003ch4\u003eStroage\u003c\/h4\u003e\u003cp\u003eUpon receipt, store at -20°C or -80°C. Avoid repeated freeze.\u003c\/p\u003e\u003ch4\u003eTarget Names\u003c\/h4\u003e\u003cp\u003eFBN1\u003c\/p\u003e\u003ch4\u003eResearch Areas\u003c\/h4\u003e\u003cp\u003eSignal transduction\u003c\/p\u003e","brand":"ABCbiolab","offers":[{"title":"Default Title","offer_id":42290999296234,"sku":"ABCCS55927","price":299.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/758-7580448_monoclonal-antibody-png-transparent-png_84100a13-a183-4937-87d3-2e6b3e98d3c9.png?v=1650882343","url":"https:\/\/danabiosci.com\/products\/fbn1-antibody-biotin-conjugated-abccs55927","provider":"Dana Bioscience","version":"1.0","type":"link"}