Neurobiology of Disease (Jun 2006)

Selective striatal neuron loss and alterations in behavior correlate with impaired striatal function in Huntington's disease transgenic rats

  • Orsolya Kántor,
  • Yasin Temel,
  • Carsten Holzmann,
  • Kerstin Raber,
  • Huu-Phuc Nguyen,
  • Chunyan Cao,
  • Hatice Özen Türkoglu,
  • Bart P.F. Rutten,
  • Veerle Visser-Vandewalle,
  • Harry W.M. Steinbusch,
  • Arjan Blokland,
  • Hubert Korr,
  • Olaf Riess,
  • Stephan von Hörsten,
  • Christoph Schmitz

Journal volume & issue
Vol. 22, no. 3
pp. 538 – 547

Abstract

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Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by selective striatal neuron loss and motor, cognitive and affective disturbances. The present study aimed to test the hypothesis of adult-onset neuron loss in striatum and frontal cortical layer V as well as alterations in behavior pointing to impaired striatal function in a recently developed transgenic rat model of HD (tgHD rats) exhibiting enlarged ventricles, striatal atrophy and pycnotic pyramidal cells in frontal cortical layer V. High-precision design-based stereological analysis revealed a reduced mean total number of neurons in the striatum but not in frontal cortical layer V of 12-month-old tgHD rats compared with age-matched wild-type controls. No alterations in mean total numbers of striatal neurons were found in 6-month-old animals. Testing 14-month-old animals in a choice reaction time task indicated impaired striatal function of tgHD rats compared with controls.

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