title: Targeting protein aggregation in neurodegeneration--lessons from polyglutamine disorders.
abstract: Polyglutamine diseases, such as Huntington's disease, are among the most common inherited neurodegenerative disorders. They share salient clinical and pathological features with major sporadic neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease and amyotropic lateral sclerosis. Over the last decade, protein aggregation has emerged as a common pathological hallmark in neurodegenerative diseases and has, therefore, attracted considerable attention as a likely shared therapeutic target. Because of their clearly defined molecular genetic basis, polyglutamine diseases have allowed researchers to dissect the relationship between neurodegeneration and protein aggregation. In this review, the authors discuss recent progress in understanding polyglutamine-mediated neurotoxicity, and discuss the most promising therapeutic strategies being developed in the polyglutamine diseases and related neurodegenerative disorders.
authors.affiliation: University of Washington Medical Center, Department of Laboratory Medicine, Box 357110, Room NW 120, Seattle, WA 98195-7110, USA.
authors.forename: Patrick
authors.initials: P
authors.lastname: Weydt
authors.forename: Albert R
authors.initials: AR
authors.lastname: La Spada
mesh_terms: D006801:Humans; D019636:Neurodegenerative Diseases / Q000188:drug therapy* / Q000378:metabolism*; D010455:Peptides / Q000378:metabolism*; D017510:Protein Folding
chemical_list: D010455:Peptides; C097188:polyglutamine