Journal article

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

A van Dellen, J Welch, RM Dixon, P Cordery, D York, P Styles, C Blakemore, AJ Hannan

NEUROREPORT | LIPPINCOTT WILLIAMS & WILKINS | Published : 2000

Abstract

Huntington's disease (HD) is an autosomal dominant condition involving progressive neurodegeneration, primarily 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 phosphoprotei..

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