Huntington's disease: animal models and transplantation repair.
Recent identification of the gene for Huntington's disease is currently attracting widespread attention. While having importance for predictive testing and the potential of elucidating the underlying disease process, this discovery does not yet provide any advances for therapeutic intervention. Here we review recent advances in the development of improved animal models of Huntington's disease and strategies for its repair. Novel toxins may better mimic the neuropathology, and provide important clues about the underlying metabolic disorder, of the human disease. In addition, recent experiments into the cellular morphology, development and function of striatal cell transplants in both rats and monkeys are now indicating the prospect of viable strategies for structural repair in this disorder.
Dunnett
S B
SB
University of Cambridge, UK.
Svendsen
C N
CN
D000818:Animals; D003342:Corpus Striatum / Q000166:cytology / Q000502:physiology; D004195:Disease Models, Animal; D015870:Gene Expression; D006801:Humans; D006816:Huntington Disease / Q000628:therapy*; D009417:Nerve Tissue / Q000637:transplantation*; D009434:Neural Pathways / Q000502:physiology