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Aging and neurodegeneration. Molecular mechanisms of neuronal loss in Huntington's disease

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Authors
Lee, Soon-Tae; Kim, Manho
Issue Date
2006-03-11
Publisher
Elsevier
Citation
Mech Ageing Dev. 2006 May;127(5):432-5. Epub 2006 Mar 9.
Keywords
*AgingAnimalsCaspases/metabolismEnzyme ActivationHumansHuntington Disease/*pathology*Nerve DegenerationNerve Tissue Proteins/geneticsNeurodegenerative Diseases/*pathologyNeurons/metabolism/*pathologyNuclear Proteins/genetics
Abstract
Huntington's disease (HD) is a fatal, genetically based late-onset neurodegenerative disorder in which a loss of neostriatal neurons is a main characteristic. The CAG trinucleotide repeat expansion encoding polyglutamine tract induces progressive deficits in intra- and inter-cellular signalling, and subsequent clinical signs developed with aging process. CAG-induced neurodegeneration and disease-onset shows aging-dependent pattern. Proposed mechanism of neurodegeneration includes intranuclear or intracellular protein aggregates, proteolytic cleavage of huntingtin (cf. caspase, calpain), altered transcription or other neurotransmitter signalling deficits. Recently, stem cell transplantation is of benefit to protect neurons against neurodegeneration and recover the functional deficit in the experimental HD model. This review focuses on current knowledge of molecular mechanisms in neurodegeneration and potential therapeutic targets in HD.
ISSN
0047-6374 (Print)
Language
English
URI
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T31-4JF8H6C-2&_user=10&_rdoc=1&_fmt=&_orig=search&_sort=d&_docanchor=&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=7e999a7bbc23fcd40f019f743bd01b64

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=16527334

http://hdl.handle.net/10371/13274
DOI
https://doi.org/10.1016/j.mad.2006.01.022
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Neurology (신경과학교실)Journal Papers (저널논문_신경과학교실)
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