N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntington's disease mice

Anton Van Dellen, John Welch, Ruth M. Dixon, Patricia Cordery, Denis York, Peter Styles, Colin Blakemore, Anthony J. Hannan*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

98 Citations (Scopus)


Huntington'S disease (HD) is an autosomal dominant condition involving progressive neurodegeneration, primarily in the corpus striatum and cerebral cortex. We have used in vivo magnetic resonance spectroscopy (MRS) to assess specific neuronal markers in transgenic mice (R6/1 line) expressing exon I of the human huntingtin gene with an expanded CAG repeat. Levels of N-Acetylaspartate (NAA), an indicator of healthy neuronal function, were significantly reduced (26%) in the corpus striatum of HD mice relative to wild-Type littermates at 5 months of age. However, levels of cholines and creatine + phosphocreatine were not altered in the HD mice. Expression of dopamine- and cAMP-Regulated phosphoprotein, 32 kDa (DARPP-32), was assessed by immunohistochemistry in the striatum of HD mice and found to be downregulated by 5 months and, even more dramatically, at 11 months of age. In contrast, expression of calbindin was not significantly decreased in HD mice. Our results suggest that the observed decreases in DARPP-32 and NAA may contribute to aberrant receptor signalling and neuronal dysfunction in HD. (C) 2000 Lippincott Williams and Wilkins.

Original languageEnglish
Pages (from-to)3751-3757
Number of pages7
Issue number17
Publication statusPublished - 27 Nov 2000
Externally publishedYes


  • Calbindin
  • DARPP-32
  • Dopamine
  • Huntington's disease
  • NAA
  • NMS
  • Polyglutamine
  • Striatum


Dive into the research topics of 'N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntington's disease mice'. Together they form a unique fingerprint.

Cite this