Medical journal of Hiroshima Universtiy Volume 53 Issue 2-4
2005-08-28 発行

進行性核上性麻痺における神経原線維変化および coiled body の出現意義 : 神経原線維変化および coiled body の ubiquitin 化に関する検討 <原著>

The Significance of Appearance of Neurofibrillary Tangles and Coiled Bodies in Progressive Supranuclear Palsy : Study about Ubiquitination of Neurofibrillary Tangles and Coiled Bodies in Progressive Supranuclear Palsy
Jin, Chenghua
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abstract
Progressive supranuclear palsy (PSP) is a degenerative disorder pathologically characterized by neuronal loss with responsive gliosis in the substantia nigra, globus pallidus, thalamus, dentate nucleus of the cerebellum and tegmentum of pons. It has been reported that abnormally phosphorylated tau in neurons and oligodendroglia forms neurofibrillary tangles (NFTs) and coiled bodies (CBs). Ubiquitin protein (Ub) plays an important role in degradation of altered protein in degenerative disorders by an ATP-dependent pathway. To clarify how the formation of NFTs and CBs in oligodendroglia is related to the formation of lesions in PSP, we classified NFTs and CBs in three PSP brains into four stages and counted the number of cells of each stage and the positive rates of Ub in total numbers of NFTs and CBs. The results showed that the appearance of NFTs was not related to neuronal loss and responsive gliosis, while CBs were closely related to neuronal loss. Although there was neuronal loss, there were few stage IV NFTs (ghost tangles) and CBs, which indicate cell death. Moreover, the positive rates of Ub in NFTs and CBs were 2.1 % and 2. 7%, respectively. These findings suggest that the formation of NFTs and CBs does not have a vital role in neuronal loss and responsive gliosis in PSP.
description
本研究の一部は科学研究費補助金,基盤研究(C) No.14570600の補助により行った。
subject
Progressive supranuclear palsy
Neurofibrillary tangles
Coiled bodies
Stage
Ubiquitin protein
rights
Copyright (c) 2005 広島大学医学出版会