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

Ina Han, Yimei You, Jeffrey H. Kordower, Scott T. Brady, Gerardo A. Morfini

Research output: Contribution to journalReview articlepeer-review

100 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1073-1091
Number of pages19
JournalJournal of Neurochemistry
Volume113
Issue number5
DOIs
StatePublished - Jun 2010
Externally publishedYes

Keywords

  • Axonal transport
  • Dying back degeneration
  • Huntingtin
  • Huntington's disease
  • Medium-sized spiny neurons

ASJC Scopus subject areas

  • Biochemistry
  • Cellular and Molecular Neuroscience

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