| Product Name | FBN1 Antibody |
|---|---|
| Description | Rabbit polyclonal antibody to FBN1 |
| Synonyms | 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 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Conjugate | Unconjugated |
| Immunogen | Recombinant Human Fibrillin-1 protein (783-850AA) |
| Isotype | IgG |
| Target | FBN1 |
| Reactivity | Human |
| Applications | ELISA, IHC |
| Form | Liquid |
| Diluent Buffer | Preservative: 0.03% Proclin 300Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Uniprot | P35555 |
| Storage | Upon receipt, store at -20°C or -80°C. Avoid repeated freeze. |
| Background | 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). |
| Supplier | Cusabio |
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