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Fibrillin-1 (FBN1) (Marfan Syndrome Marker) Antibody [FBN1/2191]

In Stock
Catalog Number Formulation Size Price
2200-MSM1-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$229.00
2200-MSM1-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$519.00
2200-MSM1-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$519.00
Flat Rate Domestic: $60 | Orders outside the US - Contact Us for Order Information | Ships next business day

Applications & Dilutions

Applications Tested Dillution Protocol Note
Immunohistochemistry (IHC)
1-2ug/ml
30 min at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95°C followed by cooling at RT for 20 minutes

Summary

Fibrillin-1 is an extracellular matrix glycoprotein that serves as a structural component of calcium-binding microfibrils. These microfibrils provide force-bearing structural support in elastic and non-elastic connective tissue throughout the body.Mutations in this gene are associated with Marfan syndrome.

Product Properties & Targets

Antibody Type
Host
Mouse
Applications
Species Reactivity
Isotype / Light Chain
IgG1 / Kappa
Cellular Localization
Extracellular matrix, Extracellular space, Secreted
Gene Name
Positive Control
HepG2 cells. Human kidney or placenta.
Immunogen
Recombinant human Fibrillin 1 fragment around aa 330-476 (Exact sequence is proprietary)
Alternate Names
Fibrillin-1, 350kDa glycoprotein component extracellular microfibril; ACMICD; FBN1; Fibrillin-15; Fibrillin-1; GPHYSD2; Marfan syndrome; MASS; MFS1; OCTD; SGS; SSKS; Weill Marchesani syndrome; WMS; WMS2

Database Links

Entrez Gene ID
SwissProt

Additional Information

Clone
FBN1/2191
Chromosome Location
15q21.1
Mol. Weight of Antigen
350kDa

Functions

  • 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).
  • Adipokine secreted by white adipose tissue that plays an important regulatory role in the glucose metabolism of liver, muscle and pancreas (PubMed:27087445, PubMed:30853600). Hormone that targets the liver in response to fasting to increase plasma glucose levels (PubMed:27087445). Binds the olfactory receptor OR4M1 at the surface of hepatocytes and promotes hepatocyte glucose release by activating the protein kinase A activity in the liver, resulting in rapid glucose release into the circulation (PubMed:27087445, PubMed:31230984). May act as a regulator of adaptive thermogenesis by inhibiting browning and energy consumption, while increasing lipid deposition in white adipose tissue (By similarity). Also acts as an orexigenic hormone that increases appetite: crosses the blood brain barrier and exerts effects on the hypothalamus (By similarity). In the arcuate nucleus of the hypothalamus, asprosin directly activates orexigenic AgRP neurons and indirectly inhibits anorexigenic POMC neurons, resulting in appetite stimulation (By similarity). Activates orexigenic AgRP neurons via binding to the olfactory receptor OR4M1 (By similarity). May also play a role in sperm motility in testis via interaction with OR4M1 receptor (By similarity).

Key References

  • Mariencheck, M.C., et al. 1995. Fibrillin-1 and fibrillin-2 show temporal and tissue-specific regulation of expression in developing elastic tissues. Connect. Tissue Res. 31: 87-97.

PubMed Links

Storage & Stability

Antibody with azide - store at 2 to 8 °C. Antibody without azide - store at -20 to -80 °C. Antibody is stable for 24 months. Non-hazardous. No MSDS required.

Limitations

This antibody is available for research use only and is not approved for use in diagnosis.

Supplied as

200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.

Warranty

There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.

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