Glyceraldehyde 3-phosphate dehydrogenase and endothelin-1 immunoreactivity is associated with cerebral white matter damage in dentatorubral-pallidoluysian atrophy.
作者： Masaki Shiozawa , Yuken Fukutani , Nobutaka Arai , Nigel J Cairns , Toshio Mizutani
DOI: 10.1046/J.1440-1789.2003.00480.X
关键词:
摘要: 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 cerebral white matter damage, especially in older cases. Intracellular accumulation protein widespread central nervous system, has been shown immobilize glyceraldehyde 3-phosphate dehydrogenase (GAPDH), which regulates glycolysis controls mRNA tissue-type plasminogen activator (tPA) tissue restoration. However, little known about pathogenesis regarding formation damage DRPLA. Therefore, pathology this was investigated examining markers related processes. Nine clinically pathologically confirmed cases were used present study. 12p all where available for genotyping (seven cases). PAS immunohistochemistry with antibodies GFAP, GAPDH endothelin-1 demonstrate astrocytosis. The polysaccharides storage state PAS-positive astrocytes detected seven GAPDH- endothelin-1-positive endothelium observed two GFAP-positivity. Based biochemical process together results, immunoreactivity associated mismetabolism may contribute
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