Volume : 50 µg
Clone Number :
Aliases : 350 kDa glycoprotein component extracell µLar 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
Product Type : polyclonal Ab Antibody
Immunogen Species : Homo sapiens (Human)
UniProt ID : P35555
Immunogen : Recombinant Human Fibrillin-1 protein (783-850AA)
Raised in : Rabbit
Species Reactivity : Human
Tested Applications :
Background : Fibrillin-1 : Structural component of the 10-12 nm diameter microfibrils of the extracell µLar matrix, which conveys both structural and reg µLatory 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 zon µLe, tendon, cornea and glomer µLus where they provide tensile strength and have anchoring roles. Fibrillin-1 also plays a key role in tissue homeostasis thro µgh 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 extracell µLar matrix protein and proteoglycan components (PubMed : 27026396). Reg µLates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively (By similarity). Negatively reg µLates 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 reg µLates 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).
Clonality : polyclonal Ab
Isotype : IgG
Purification Method : >95%, Protein G purified
Conj µgate : FITC
Buffer : Preservative : 0.03% Proclin 300
Constituents : 50% Glycerol, 0.01M PBS, pH 7.4
Form : Liquid
Stroage : Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Target Names : FBN1
Research Areas : Signal transduction