title: Ubiquitin and ubiquitin-related proteins in the brains of patients with atypical Pick's disease without Pick bodies and dementia with motor neuron disease.
abstract: Nine cases of atypical Pick's disease without Pick bodies (aPiD) and seven cases of dementia with motor neuron diseases (D-MND) were compared using immunohistochemistry of ubiquitin (ub) and ub-related proteins. All cases showed rostral-dominant atrophy in the temporal and frontal lobes, although the degree of atrophy with neuronal loss was much more severe in the aPiD cases. In both aPiD and D-MND cases, ub-positive and tau-negative structures were found mainly in the hippocampal dentate gyrus and cerebral cortex. Granular cells of the dentate gyrus showed similar ub-positive intraneuronal inclusions in both cases. In the aPiD cases, most of the ub-positive cortical structures were ub-positive dendrites in layers II-IIIab and layers V-VI, although some neurons also showed diffuse ub-positive staining in the cytoplasm. In the D-MND cases, some neurons showed ub-positive inclusions in layers II-IIIab, and ub-positive dendrites were unremarkable. The number of ub-positive neurons and dendrites in relation to the degree of neuronal loss in the cerebral cortex were then evaluated. The number of ub-positive neurons in the regions showing very mild to mild neuronal loss was significantly greater in the D-MND cases than in the aPiD cases. However, in the aPiD cases, the number of ub-positive neurons was significantly greater in the regions showing moderate neuronal loss. When double-immunostained, almost all ub-positive structures were positive for ub-binding protein p62. Some ub-positive or negative neurons in the cerebral cortex were immunostained with anti-ub ligase (Parkin) and anti-ub C-terminal hydrolase (UCH-L1) antibodies. Granular cells of the dentate gyrus were weakly positive for UCH-L1. There could be some differences in the mechanism by which neurons in the cerebral cortex accumulate ub between aPiD and D-MND.
authors.affiliation: Department of Psychiatry, Yokohama City University, Yokohama, Japan.
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Furukawa
authors.forename: Eizo
authors.initials: E
authors.lastname: Iseki
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Hino
authors.forename: Toshinari
authors.initials: T
authors.lastname: Odawara
authors.forename: Kenji
authors.initials: K
authors.lastname: Ikeda
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Kenji
authors.initials: K
authors.lastname: Kosaka
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000378:metabolism* / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000378:metabolism* / Q000473:pathology*; D020774:Pick Disease of the Brain / Q000378:metabolism* / Q000473:pathology*; D025841:Small Ubiquitin-Related Modifier Proteins / Q000032:analysis*; D025801:Ubiquitin / Q000032:analysis*
chemical_list: D025841:Small Ubiquitin-Related Modifier Proteins; D025801:Ubiquitin