Protein aggregation and neurodegenerative disease.

作者: Christopher A Ross , Michelle A Poirier

DOI: 10.1038/NM1066

关键词: Protein foldingProtein structureAmyloidAmyotrophic lateral sclerosisNeuroscienceProtein aggregationDiseaseVirologyHuntingtin ProteinBiologyPathogenesis

摘要: Neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's (PD), Huntington's (HD), amyotrophic lateral sclerosis (ALS) and prion are increasingly being realized to have common cellular molecular mechanisms including protein aggregation inclusion body formation. The aggregates usually consist of fibers containing misfolded with a beta-sheet conformation, termed amyloid. There is partial but not perfect overlap among the cells in which abnormal proteins deposited that degenerate. most likely explanation inclusions other visible represent an end stage cascade several steps, earlier steps may be more directly tied pathogenesis than themselves. For diseases, genetic variants assist explaining sporadic forms developing mouse models. now increased understanding pathways involved aggregation, some recent clues emerged toxicity. These leading approaches toward rational therapeutics.

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