Selective striatal neuron loss and alterations in behavior correlate with impaired striatal function in Huntington's disease transgenic rats.
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.
Department of Anatomy and Cell Biology, RWTH Aachen University, Wendlingweg 2, 52074 Aachen, Germany.
Orsolya
O
Kántor
Yasin
Y
Temel
Carsten
C
Holzmann
Kerstin
K
Raber
Huu-Phuc
HP
Nguyen
Chunyan
C
Cao
Hatice Ozen
HO
Türkoglu
Bart P F
BP
Rutten
Veerle
V
Visser-Vandewalle
Harry W M
HW
Steinbusch
Arjan
A
Blokland
Hubert
H
Korr
Olaf
O
Riess
Stephan
S
von Hörsten
Christoph
C
Schmitz
D000818:Animals; D030801:Animals, Genetically Modified; D001522:Behavior, Animal / Q000502:physiology*; D002452:Cell Count; D003342:Corpus Striatum / Q000166:cytology / Q000473:pathology*; D004195:Disease Models, Animal; D006816:Huntington Disease / Q000473:pathology*; D007150:Immunohistochemistry; D009474:Neurons / Q000166:cytology / Q000473:pathology*; D051381:Rats; D017207:Rats, Sprague-Dawley