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UCH L1 and neurodegeneration Rieko Setsuie 1 , Hitoshi Osaka 2 , Keiji Wada 1 1Department of Degenerative Neurological Diseases, National Institute of Neuroscience, NCNP 2Divisions of Neurology, Kanagawa Children's Medical Center Keyword: ユビキチン・プロテアソーム系 , パーキンソン病 , gracile axonal dystrophy(gad)mouse , UCH L1 pp.63-73
Published Date 2004/2/10
DOI https://doi.org/10.11477/mf.1431100180
  • Abstract
  • Look Inside

 Deubiquitiytlating enzymes reversibly control the state of ubiquitylation of proteins. Ubiquitin carboxy terminal hydrolase L1(UCH L1)is one of the deubiqitylating enzymes and exclusively expressed in neurons and testis/ovary. UCH L1 is also found in the cellular aggregates from the brain with neurodegenerative diseases such as Parkinson's disease and Alzheimer's disease. UCH L1 was reported to be a causal gene for the familial parkinson's disease and the gracile axonal dystrophy(gad)mouse. These facts suggest that the function of UCH L1 is closely related to the survival and maintenance of the neuron. In this review, we would like to focus on the recent findings on the UCH L1 function and its relation to neurodegeneration.


Copyright © 2004, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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