Anti-Fibrillin (Pan) Antibody, clone 689

Descrição detalhada : Fibrillins are glycosolated and disulfide-rich components of the extracellular matrix belonging to the Fibrillin-LTBP protein family. They serve an important function in both nonelastic and elastic tissues such as eye, bone and blood vessels. The functional units of Fibrillin are known as microfibrils, multiprotein assemblies, which act as structural scaffolding for the limiting of the tissue elasticity, structural integrity of different organ systems, the assembly of elastic fibers, and regulation of BMP growth factor signaling. There are three known isoforms; Fibrillin 1, Fibrillin 2, and Fibrillin 3. Fibrillin 3 is the most recently described member of the Fibrillin family, with highest expression in the brain. Alterations in the microfibril assembly often occur in patients with fibrillinopathies such as; Congenital Contractual Arachnodactyly (CCA) which results in disruption of Fibrillin 2, and Marfan's syndrome resulting from Fibrillin 1 mutation.
Sinónimos : EGF-containing fibulin-like extracellular matrix protein 1, Fibrillin-like protein, Extracellular protein S1-5
Armazenamento : +2°C to +8°C
Embalagem : 1X1EA