Hereditary dentatorubral-pallidoluysian atrophy: ubiquitinated filamentous inclusions in the cerebellar dentate nucleus neurons
作者： Yasuko Hayashi , Akiyoshi Kakita , Mitsunori Yamada , Shigekimi Egawa , Shinsaku Oyanagi
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摘要: We examined the cerebellar dentate nucleus (CDN) in 16 patients with hereditary dentatorubral-pallidoluysian atrophy (DRPLA), one of neurodegenerative diseases caused by expansion a CAG repeat encoding polyglutamine tract disease protein. In all patients, some CDN neurons were found to contain ubiquitinated filamentous inclusions their cytoplasm. On hematoxylin and eosin preparations, these eosinophilic, basophilic or amphophilic, often areas pale Electron microscopy revealed that they consisted bundles filaments somewhat thicker than neurofilaments. These features present indistinguishable from those skein-like (SLI) previously described lower motor sporadic amyotrophic lateral sclerosis. conclude SLI can also occur DRPLA believe reflect characteristic pathological process this disease.
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