Document Detail

Cell therapy in Huntington disease.
MedLine Citation:
PMID:  18341412     Owner:  NLM     Status:  MEDLINE    
Huntington disease (HD), caused by polyglutamate expansions in the huntingtin protein, is a progressive neurodegenerative disease resulting in cognitive and motor impairments and death. Neuronal dysfunction and degeneration contribute to progressive physiological, motor, cognitive, and emotional disturbances characteristic of HD. A major impetus for research into the treatment of HD has centered on cell therapy strategies to protect vulnerable neuronal cell populations or to replace dysfunctional or dying cells. The work underlying 3 approaches to HD cell therapy includes the potential for self-repair through the manipulation of endogenous stem cells and/or neurogenesis, the use of fetal or stem cell transplantation as a cell replacement strategy, and the administration of neurotrophic factors to protect susceptible neuronal populations. These approaches have shown some promising results in animal models of HD. Although striatal transplantation of fetal-derived cells has undergone clinical assessment since the 1990s, many cell therapy strategies have yet to be applied in the clinic environment. A more thorough understanding of the pathophysiologies underlying HD as well as the response of both endogenous and exogenous cells to the degenerating brain will inform their merit as potential therapeutic agents and enhance the framework by which the success of such strategies are determined.
Claire D Clelland; Roger A Barker; Colin Watts
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Neurosurgical focus     Volume:  24     ISSN:  1092-0684     ISO Abbreviation:  Neurosurg Focus     Publication Date:  2008  
Date Detail:
Created Date:  2008-03-17     Completed Date:  2008-04-17     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  100896471     Medline TA:  Neurosurg Focus     Country:  United States    
Other Details:
Languages:  eng     Pagination:  E9     Citation Subset:  IM    
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MeSH Terms
Cell- and Tissue-Based Therapy / methods*,  trends
Huntington Disease / therapy*
Grant Support
G9828345//Medical Research Council

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