GFAP and its role in Alexander disease

Academic Article


  • 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 αB-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 with other intermediate filament diseases and assess potential therapies as part of this review as well as emerging trends in disease diagnosis and other aspects concerning GFAP. © 2007 Elsevier Inc. All rights reserved.
  • Authors

    Published In

    Digital Object Identifier (doi)

    Author List

  • Quinlan RA; Brenner M; Goldman JE; Messing A
  • Start Page

  • 2077
  • End Page

  • 2087
  • Volume

  • 313
  • Issue

  • 10