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Cellular and molecular mechanisms implicated in pathogenesis of selective neurodegeneration in Huntington＇s disease

Frontiers of physics 2012年第5期7卷 459-476页

作者： Jeffrey R CANTLE Center for Neurobehavioral Genetics Semel Institute for Neuroscience & Human Behavior David Geffen School of Medicine at UCLA_ University of California Los Angeles CA 90095 USA DepartmentofPsychiatry & Biobehavioral Sciences David Geffen School of Medicine at UCLA University of California Los Angeles CA 90095 USA Brain Research Institute. David Geffen School of Medicine at UCLA University of California Los Angeles CA 90095 USA Interdepartmental Program for Neuroscience David Geffen School of Medicine at UCLA University of CaliJbrnia Los Angeles CA 90095 USA

Huntington＇s disease （HD） is one of the most common dominantly-inherited neurodegenerative disorders and is caused by a CAG repeat expansion in the huntingtin gene. HD is characterized by selective degeneration of subpopulations of neurons in the brain, however the precise underlying mechanisms how a ubiquitously expressed disease protein could target specific types of neurons for degeneration remains a critical, yet unanswered question for HD and other major neurodegenerative disorders. In this review, we describe the expanding view of selective neuronal vulnerability in HD, based on recent neuropathological and neuroimaging studies. We will also summarize the systematic effort to define the cell types in which mutant Huntingtin expression is critical for pathogenesis of vulnerable neurons in the striatum and cortex. Finally, we will describe selected, emerging molecular mechanisms that are implicated in selective disease processes in HD. Together, the field has begun to appreciate the distinct molecular pathogenic roles of mutant huntingtin in different cell types that may contribute to the selective neuronal vulnerability, with dissection of such mechanisms likely to yield novel molecular targets for HD therapy.

关键词： Huntington's disease neurodegeneration selective neuronal vulnerability cortex striatum conditional mouse model cell-autonomous toxicity pathological cell-cell interaction pathogenesis therapeutic targets

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