Suppression of GFAP toxicity by alphaB-crystallin in mouse models of Alexander disease.

@article{Hagemann2009SuppressionOG,
  title={Suppression of GFAP toxicity by alphaB-crystallin in mouse models of Alexander disease.},
  author={Tracy L. Hagemann and Wilbert C. Boelens and Eric Wawrousek and Albee Messing},
  journal={Human molecular genetics},
  year={2009},
  volume={18 7},
  pages={1190-9}
}
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 elevation of Cryab is a consistent feature of AxD; however, its role in Rosenthal… CONTINUE READING

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