The Alexander Disease–Causing Glial Fibrillary Acidic Protein Mutant, R416W, Accumulates into Rosenthal Fibers by a Pathway That Involves Filament Aggregation and the Association of αB-Crystallin and HSP27

Here, we describe the early events in the disease pathogenesis of Alexander disease. This is a rare and usually fatal neurodegenerative disorder whose pathological hallmark is the abundance of protein aggregates in astrocytes. These aggregates, termed “Rosenthal fibers,” contain the protein chaperon...

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Main Authors: Perng, Ming Der, Su, Mu, Wen, Shu Fang, Li, Rong, Gibbon, Terry, Prescott, Alan R., Brenner, Michael, Quinlan, Roy A.
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society of Human Genetics 2006
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Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1559481/
https://ncbi.nlm.nih.gov/pubmed/16826512
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