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hypothesis
Nature 412, 143-144 (12 July 2001) | doi:10.1038/35084141
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Academic Neuropathologist
- University Hospitals Case Medical Center
- Cleveland, Ohio, USA
Professor
- University of Cincinnati and Cincinnati Children's Research Foundation
- Cincinnati, OH
Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats
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Abstract
Neurodegenerative diseases resulting from expanded repeat sequences of glutamine residues are associated with the formation of protein aggregates in the cell nuclei of the affected neurons, but whether these are pathogenic is controversial. Recent observations indicate that the ages of onset of these diseases are exponential functions of the repeat lengths and that the probability of neural death is constant with time. The only process known to us that could give rise to such behaviour is nucleation of the aggregates.
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