Pathology of Basal Ganglia in Neurodegenerative Diseases Koichi Wakabayashi 1 , Kunikazu Tanji 1 , Fumiaki Mori 1 1Department of Neuropathology,Institute of Brain Science,Hirosaki University Graduate School of Medicine Keyword: tauopathy , α-synucleinopathy , TDP-43 proteinopathy , basal ganglia , pathology pp.429-439
Published Date 2009/4/1
DOI https://doi.org/10.11477/mf.1416100468
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 Intra- and/or extracellular proteinaceous inclusions in the brain tissue are characteristic pathological markers of many neurodegenerative diseases. Tau protein in neurofibrillary tangles and β-amyloid in senile plaques are associated with Alzheimer's disease. Tau is associated with various neurological conditions,which are collectively referred to as tauopathies. α-Synucleinopathy is a term that collectively refers to a set of diseases in which neurodegeneration is accompanied by intracellular accumulation of α-synuclein in neurons or glial cells. Recently,TDP-43 has been identified as a major disease protein in the ubiquitinated inclusions in deseases such as amyotrophic lateral sclerosis and frontotemporal lobar degeneration with tau-negative,ubiquitin-positive inclusions. Thus,these neurodegenerative disorders comprise a new disease class,namely,TDP-43 proteinopathy. In this article,we review the present understanding of histopathological features of basal ganglia lesions in protein conformation disorders,including tauopathy,α-synucleinopathy,and TDP-43 proteinopathy.

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