Fibrillin 1 (FBN1) is a 350kDa glycoprotein and a major constituent of microfibrils. The gene encoding it is localized on human chromosome 15. FBN1, which is a 2871 amino acid protein, has seven transforming growth factor-ß1 binding protein-like (TB) domains and epidermal growth factor-like (EGF) domains.
Synonyms: Anti-Fibrillin-1 precursor
Storage: -20C
Application: Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.Immunohistochemistry (1 paper)
Biochem Physiol Actions: Fibrillin 1 (FBN1) is an extracellular regulator of transforming growth factor ß-1 (TGF-ß1). It is a cytokine which is involved in the transdifferentiation of fibroblasts into myofibroblasts and also regulates microfibril assembly and stability. Mutations in the gene encoding FBN1 are known to be associated with Marfan syndrome (MFS).
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