GFAP and its role in Alexander disease.

  title={GFAP and its role in Alexander disease.},
  author={Roy A Quinlan and Michael Brenner and James E. Goldman and Albee Messing},
  journal={Experimental cell research},
  volume={313 10},
Here we review how GFAP mutations cause Alexander disease. The current data suggest that a combination of events cause the disease. These include: (i) the accumulation of GFAP and the formation of characteristic aggregates, called Rosenthal fibers, (ii) the sequestration of the protein chaperones alpha B-crystallin and HSP27 into Rosenthal fibers, and (iii) the activation of both Jnk and the stress response. These then set in motion events that lead to Alexander disease. We discuss parallels… CONTINUE READING
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