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Oligodendrocytic polyglutamine pathology in dentatorubral‐pallidoluysian atrophy
Author(s) -
Yamada Mitsunori,
Sato Toshiya,
Tsuji Shoji,
Takahashi Hitoshi
Publication year - 2002
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.10352
Subject(s) - atrophy , pathology , pathological , pathogenesis , biology , degenerative disease , medicine , disease
White matter degeneration is one of the pathological conditions of dentatorubral‐pallidoluysian atrophy. Autopsy brains exhibited a reduced number of glial cells in the lesions and an involvement of oligodendrocytes in nuclear inclusion formation, which previously has been recognized only as a pathological hallmark in neurons. Dentatorubral‐pallidoluysian atrophy transgenic mice showed an increased number of affected glias with increasing age and with larger expansions of CAG repeats. These findings suggest that glial cells in dentatorubral‐pallidoluysian atrophy also are involved in the polyglutamine pathogenesis.