Document Detail

Striosome-matrix pathology and motor deficits in the YAC128 mouse model of Huntington's disease.
MedLine Citation:
PMID:  18809498     Owner:  NLM     Status:  MEDLINE    
Huntington's disease is characterized by striatal degeneration and progressive motor deficits. To examine striatal compartment-specific pathology and its relation to motor symptoms, we used immunohistochemistry to identify and measure the striosomes and matrix of 7-13-month-old YAC128 and wild type (WT) mice that were previously tested on motor tasks. Compared to WTs, 13-month-old YAC128s showed volume shrinkage in striosomes, and cell loss in both compartments. The percent cell loss was greater in striosomes than matrix. Striosome volume and cell loss was greatest in the dorsolateral striatum. YAC128 rotarod and balance beam deficits preceded volume and cell loss. At 13 months, YAC128 balance beam slips and striosome cell number were inversely correlated. The results show that pathology in older YAC128s manifests as an abnormal striosome to matrix ratio and suggest that this imbalance can contribute to some motor symptoms.
Collene Lawhorn; Diane M Smith; Lucy L Brown
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2008-08-30
Journal Detail:
Title:  Neurobiology of disease     Volume:  32     ISSN:  1095-953X     ISO Abbreviation:  Neurobiol. Dis.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-11-28     Completed Date:  2009-01-28     Revised Date:  2014-09-05    
Medline Journal Info:
Nlm Unique ID:  9500169     Medline TA:  Neurobiol Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  471-8     Citation Subset:  IM    
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MeSH Terms
Analysis of Variance
Cell Death
Corpus Striatum / pathology*
Disease Models, Animal*
Huntington Disease / pathology*,  physiopathology*
Mice, Transgenic
Motor Activity* / physiology
Grant Support
F31 NS055592/NS/NINDS NIH HHS; F31 NS055592-02/NS/NINDS NIH HHS; R01 21356//PHS HHS

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