GFAP

IgG1. k

Purified Glial Fibrillary Acidic Protein (GFAP)

Monoclonal Antibodies to Cytoskeletal Filaments and Interstitial Proteins

10mM Phosphate pH 7. 4 containing 150mM Sodium chlorideApplications Notes : Suitable for use in immunohistochemistry. Each laboratory should determine an optimum working titer for use in its particular application. Other applications have not been tested but use in such assays should not necessarily be excluded.

GFAP a class-III intermediate filament is a cell-specific marker that during the development of the central nervous system distinguishes astrocytes from other glial cells.

Cytoplasm. Note=Associated with intermediate filaments.

Defects in GFAP are a cause of Alexander disease [MIM:203450]. Alexander disease is a rare disorder of the central nervous system. It is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of Rosenthal fibers which are cytoplasmic inclusions in astrocytes. The most common form affects infants and young children and is characterized by progressive failure of central myelination usually leading to death usually within the first decade. Infants with Alexander disease develop a leukoencephalopathy with macrocephaly seizures and psychomotor retardation. Patients with juvenile or adult forms typically experience ataxia bulbar signs and spasticity and a more slowly progressive course.