Article
Suppression of GFAP toxicity by alphaB-crystallin in mouse models of Alexander disease.
Human molecular genetics - 1 Apr 2009
Hagemann Tracy L, Boelens Wilbert C, Wawrousek Eric F, Messing Albee
Abstract excerpt
Alexander disease (AxD) is a primary disorder of astrocytes caused by dominant mutations in the gene for glial fibrillary acidic protein (GFAP). These mutations lead to protein aggregation and formation of Rosenthal fibers, complex astrocytic inclusions that contain GFAP, vimentin, plectin, ubiquitin, Hsp27 and alphaB-crystallin. The small heat shock protein alphaB-crystallin (Cryab) regulates GFAP assembly, and...
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