GFAP and its role in Alexander disease
RA Quinlan, M Brenner, JE Goldman… - Experimental cell research, 2007 - Elsevier
RA Quinlan, M Brenner, JE Goldman, A Messing
Experimental cell research, 2007•ElsevierHere 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 …
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 …
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.
Elsevier