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J Neurochem. 2010 Jun;113(5):1073-91. doi: 10.1111/j.1471-4159.2010.06672.x. Epub 2010 Mar 17.

Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features.

Author information

1
Department of Anatomy and Cell Biology. University of Illinois at Chicago, Chicago, Illinois 60612, USA.

Abstract

Abnormal expansion of a polyglutamine tract in huntingtin (Htt) protein results in Huntington's disease (HD), an autosomal dominant neurodegenerative disorder involving progressive loss of motor and cognitive function. Contrasting with the ubiquitous tissue expression of polyglutamine-expanded Htt, HD pathology is characterized by the increased vulnerability of specific neuronal populations within the striatum and the cerebral cortex. Morphological, biochemical, and functional characteristics of neurons affected in HD that might render these cells more vulnerable to the toxic effects of polyglutamine-Htt are covered in this review. The differential vulnerability of neurons observed in HD is discussed in the context of various major pathogenic mechanisms proposed to date, and in line with evidence showing a 'dying-back' pattern of degeneration in affected neuronal populations.

PMID:
20236390
PMCID:
PMC2890032
DOI:
10.1111/j.1471-4159.2010.06672.x
[Indexed for MEDLINE]
Free PMC Article

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