Anti-Fibrillin monoclonal antibody (DMAB8368)
Mouse Anti-Bovine Fibrillin monoclonal antibody for IHC, WB
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Microfibrils from zonular apparatus of bovine eye
FBN; SGS; WMS; MASS; MFS1; OCTD
FBN1 (Fibrillin 1) is a Protein Coding gene. Diseases associated with FBN1 include stiff skin syndrome and marfan syndrome. Among its related pathways are Degradation of the extracellular matrix and ERK Signaling. GO annotations related to this gene include calcium ion binding and extracellular matrix structural constituent. An important paralog of this gene is LTBP3. This gene encodes a member of the fibrillin family of proteins. The encoded preproprotein is proteolytically processed to generate two proteins including the extracellular matrix component fibrillin-1 and the protein hormone asprosin. 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 nonelastic connective tissue throughout the body. Asprosin, secreted by white adipose tissue, has been shown to regulate glucose homeostasis. Mutations in this gene are associated with Marfan syndrome and the related MASS phenotype, as well as ectopia lentis syndrome, Weill-Marchesani syndrome, Shprintzen-Goldberg syndrome and neonatal progeroid syndrome.
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Charbonneau, NL; Jordan, CD; et al. Microfibril Structure Masks Fibrillin-2 in Postnatal Tissues. JOURNAL OF BIOLOGICAL CHEMISTRY 285:20242-20251(2010).
Guo, G; Munoz-Garcia, B; et al. Antagonism of GxxPG fragments ameliorates manifestations of aortic disease in Marfan syndrome mice. HUMAN MOLECULAR GENETICS 22:433-443(2013).
Custom Antibody Labeling
We offer labeled antibodies using our catalogue antibody products and a broad range of intensely fluorescent dyes and labels including HRP, biotin, ALP, Alexa Fluor® dyes, DyLight® Fluor dyes, R-phycoerythrin (R-PE), at scales from less than 100 μg up to 1 g of IgG antibody.