Oligodendrocytic polyglutamine pathology in dentatorubral-pallidoluysian atrophy.
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.
Yamada
Mitsunori
M
Department of Pathology, Brain Research Institute, Niigata University, 1 Asahimachi, Niigata 951-8585, Japan. nori@bri.niigata-u.ac.jp
Sato
Toshiya
T
Tsuji
Shoji
S
Takahashi
Hitoshi
H
D000328:Adult; D000368:Aged; D000818:Animals; D001921:Brain / Q000378:metabolism* / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics; D008875:Middle Aged; D020191:Myoclonic Epilepsies, Progressive / Q000378:metabolism* / Q000473:pathology*; D009836:Oligodendroglia / Q000378:metabolism*; D010455:Peptides / Q000378:metabolism*
D010455:Peptides; C097188:polyglutamine