Title : [Polyglutamine diseases: a pathologic view].

Pub. Date : 2003 Nov

PMID : 15152499






2 Functional Relationships(s)
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1 It has been widely accepted that there is a discrepancy between clinical features and lesion distribution evaluated by neuronal loss in the polyglutamine diseases including Huntington"s disease and dentatorubral-pallidoluysian atrophy (DRPLA). polyglutamine atrophin 1 Homo sapiens
2 In a study of DRPLA brains, we found that intranuclear diffuse accumulation of mutant proteins with expanded polyglutamine stretches is another pathology involving many neurons in various nervous system regions including the dentatorubral and pallidoluysian systems, in which the number of affected neurons was constantly high. polyglutamine atrophin 1 Homo sapiens