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.
Kántor
Orsolya
O
Department of Anatomy and Cell Biology, RWTH Aachen University, Wendlingweg 2, 52074 Aachen, Germany.
Temel
Yasin
Y
Holzmann
Carsten
C
Raber
Kerstin
K
Nguyen
Huu-Phuc
HP
Cao
Chunyan
C
Türkoglu
Hatice Ozen
HO
Rutten
Bart P F
BP
Visser-Vandewalle
Veerle
V
Steinbusch
Harry W M
HW
Blokland
Arjan
A
Korr
Hubert
H
Riess
Olaf
O
von Hörsten
Stephan
S
Schmitz
Christoph
C
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