Anti-Fibrillin monoclonal antibody (DMAB8368)

Mouse Anti-Bovine Fibrillin monoclonal antibody for IHC, WB Datasheet

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Host Species
Antibody Isotype
Species Reactivity
Microfibrils from zonular apparatus of bovine eye


Alternative Names
Entrez Gene ID
UniProt ID

Product Background

Gene summary
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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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).
Matt, P; Habashi, J; et al. Recent advances in understanding Marfan syndrome: Should we now treat surgical patients with losartan?. JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 135:389-394(2008).

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