ITA0334
ITA0334
- Catalog: ITA0334
- Gene/Protein: FBN1
- Product Description: Immunotag™ Fibrillin-1 Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet |
|
| Target Protein | Fibrillin-1 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | IHC,ELISA |
| Recommended Dilution | IHC 1:50-1:200 |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat |
| Host Species | Rabbit |
| Immunogen | A synthesized peptide derived from human Fibrillin-1 |
| Specificity | Fibrillin-1 Antibody detects endogenous levels of Fibrillin-1 |
| Purification | The antiserum was purified by peptide affinity chromatography. |
| Form | Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt |
| Gene Name | FBN1 |
| Accession No. | P35555 |
| Alternate Names | 350 kDa glycoprotein component extracellular microfibril; ACMICD; FBN 1; FBN; FBN1; FBN1_HUMAN; Fibrillin 15; Fibrillin-1; Fibrillin1; Fibrillin15; GPHYSD2; Marfan syndrome; MASS; MFS 1; MFS1; OCTD; SGS; SSKS; Weill Marchesani syndrome; WMS; WMS2; |
| Description | 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). |
| Cell Pathway/ Category | Primary Polyclonal Antibody |
| Protein MW | 312kDa |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
| English_US |
|
| Danish |
|
| Dutch |
|
| English_UK |
|
| French |
|
| German |
|
| Spanish |
|
| Norwegian |
|
| Portuguese |
|
| Finnish |
|
| Swedish |
|
| Polish |
|