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

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.

journal_name

J Neurochem

authors

Han I,You Y,Kordower JH,Brady ST,Morfini GA

doi

10.1111/j.1471-4159.2010.06672.x

subject

Has Abstract

pub_date

2010-06-01 00:00:00

pages

1073-91

issue

5

eissn

0022-3042

issn

1471-4159

pii

JNC6672

journal_volume

113

pub_type

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