Article
Glyceraldehyde 3-phosphate dehydrogenase and endothelin-1 immunoreactivity is associated with cerebral white matter damage in dentatorubral-pallidoluysian atrophy.
Neuropathology : official journal of the Japanese Society of Neuropathology - 1 Mar 2003
Shiozawa Masaki, Fukutani Yuken, Arai Nobutaka, Cairns Nigel J, Mizutani Toshio, Isaki Kiminori, Lantos Peter L, Wada Yuji
Abstract excerpt
DRPLA is a rare neurodegenerative disorder caused by CAG triplet elongation on chromosome 12p. In addition to neurodegeneration of both the dentatorubral and pallidoluysian systems, there is cerebral white matter damage, especially in older cases. Intracellular accumulation of DRPLA protein is widespread in the central nervous system, and DRPLA protein has been shown to immobilize glyceraldehyde 3-phosphate...
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