Alexander disease (AxD) is a neurodegenerative disorder with prominent white matter degeneration and the presence of Rosenthal fibers containing aggregates of glial fibrillary acidic protein (GFAP), and small stress proteins HSP27 and αB-crystallin, and widespread reactive gliosis.
The alpha B-crystallin and hsp27 polypeptide isoform patterns of Alexander disease CNS are also distinct from those of control samples, suggesting that postranslational modifications may be involved in Rosenthal fiber formation.
Our results suggest that astrocytes in Alexander's disease may be involved in an as yet unknown kind of stress reaction that causes the accumulation of alpha B-crystallin and HSP27 and results in Rosenthal fiber formation.