Synergistic effects of the SAPK/JNK and the proteasome pathway on glial fibrillary acidic protein (GFAP) accumulation in Alexander disease
G Tang, Z Xu, JE Goldman - Journal of biological chemistry, 2006 - ASBMB
Protein aggregates in astrocytes that contain glial fibrillary acidic protein (GFAP), small heat
shock proteins, and ubiquitinated proteins are termed Rosenthal fibers and characterize
Alexander disease, a leukodystrophy caused by heterozygous mutations in GFAP. The
mechanisms responsible for the massive accumulation of GFAP in Alexander disease
remain unclear. In this study, we show that overexpression of both wild type and R239C
mutant human GFAP led to cytoplasmic inclusions. GFAP accumulation also led to a …
shock proteins, and ubiquitinated proteins are termed Rosenthal fibers and characterize
Alexander disease, a leukodystrophy caused by heterozygous mutations in GFAP. The
mechanisms responsible for the massive accumulation of GFAP in Alexander disease
remain unclear. In this study, we show that overexpression of both wild type and R239C
mutant human GFAP led to cytoplasmic inclusions. GFAP accumulation also led to a …
