FBN1 / Fibrillin 1 Antibody (clone 3H6)
Mouse Monoclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| WB, IHC-P, E |
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Primary Accession | P35555 |
Reactivity | Human |
Host | Mouse |
Clonality | Monoclonal |
Clone Names | 3H6 |
Calculated MW | 312kDa |
Dilution | IHC-P (5 µg/ml), WB (1:500-1:1000), |
Gene ID | 2200 |
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Other Names | Fibrillin-1, FBN1, FBN |
Target/Specificity | Human Fibrillin 1 |
Reconstitution & Storage | Aliquot and store at -20°C or -80°C. Avoid freeze-thaw cycles. |
Precautions | FBN1 / Fibrillin 1 Antibody (clone 3H6) is for research use only and not for use in diagnostic or therapeutic procedures. |
Name | FBN1 (HGNC:3603) |
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Synonyms | FBN |
Function | [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 (PubMed:27026396). 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 (PubMed:27026396). 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 (PubMed:27026396). 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 (PubMed:24039232). This leads to disruption of TNFSF11-induced Ca(2+) 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). |
Cellular Location | Secreted. Note=Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin (PubMed:24982166) [Asprosin]: Secreted. Note=Secreted by white adipose tissue and circulates in the plasma. |
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Provided below are standard protocols that you may find useful for product applications.
Background
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1- containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.
References
Pereira L.V.,et al.Hum. Mol. Genet. 2:961-968(1993).
Uyeda T.,et al.J. Hum. Genet. 49:404-407(2004).
Rieder M.J.,et al.Submitted (SEP-2009) to the EMBL/GenBank/DDBJ databases.
Zody M.C.,et al.Nature 440:671-675(2006).
Mural R.J.,et al.Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases.
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