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Huntington Disease Pathology Unfolds

Courtesy: Larry Marsh and Judit Pallos  IN A FLY'S EYE: The regular repeating structure of the Drosophila melanogaster compound eye is disrupted when polyglutamine (polyQ 108) is expressed, making the fly eye an excellent model for huntingtin-derived pathology. Dissecting the mechanism of neuronal demise in Huntington disease (HD) has had its share of twists and turns. It took a decade to go from marker1 to gene,2 and now, after another decade, the details are beginning to come together.

Ricki Lewis
Courtesy: Larry Marsh and Judit Pallos
 IN A FLY'S EYE: The regular repeating structure of the Drosophila melanogaster compound eye is disrupted when polyglutamine (polyQ 108) is expressed, making the fly eye an excellent model for huntingtin-derived pathology.

Dissecting the mechanism of neuronal demise in Huntington disease (HD) has had its share of twists and turns. It took a decade to go from marker1 to gene,2 and now, after another decade, the details are beginning to come together. Three papers published in 2001 focus on two key roles for the pathological form of huntingtin protein--interfering with transcription3,4 and plugging up proteasomes, the cellular trash compactors for misfolded proteins.5 While different, these hypotheses are not mutually exclusive, and may point to multiple effects that inevitably cause the devastating symptoms of HD.

Data derived from the Science Watch/Hot Papers database and the Web of Science (ISI, Philadelphia) show...

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