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Vol. 16, No. 11, pp. 1307-1313, June 1, 2002
Skirball Institute of Biomolecular Medicine, Departments of Medicine and Cell Biology, New York University School of Medicine, New York, New York 10016, USA
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Introduction |
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An important subgroup of human neurodegenerative
diseases is associated with abnormal expansions of glutamine repeats
found in several otherwise unrelated proteins (Zoghbi and Orr 2000
). Interest in these polyglutamine diseases is fueled both by their clinical significance and by the belief that lessons gleaned from these
relatively rare conditions will apply to other more prevalent human
neurodegenerative disorders and perhaps more generally to other
diseases of aging. The basis for this belief is the observation that
polyglutamine diseases and common neurodegenerative disorders, such as
Alzheimer's disease and Parkinson's disease, share as a common
feature the accumulation of abnormal protein
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