Document Detail

Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy.
MedLine Citation:
PMID:  8255479     Owner:  NLM     Status:  MEDLINE    
The Huntington's disease (HD) gene mutation has recently been found; however, the biochemical defect that leads to neurodegeneration is still unknown. A progressive impairment of neuronal energy metabolism is a possible etiologic factor. We tested this possibility using localized proton nuclear magnetic resonance (NMR) spectroscopy in 18 patients at high risk for, or suffering from, HD as compared with normal controls. Lactate concentrations were increased in the occipital cortex of symptomatic HD patients when compared with normal controls, and the lactate level correlated with duration of illness. In addition, several patients showed highly elevated lactate levels in the basal ganglia. Basal ganglia levels of N-acetylaspartate were lowered and choline dramatically elevated, relative to creatine, reflecting neuronal loss and gliosis in this brain region. These findings are consistent with a possible defect in energy metabolism in HD, which could contribute to the pathogenesis of the disease. The presence of elevated lactate in HD brains may provide a simple marker that can be followed over time noninvasively and repeatedly to aid in devising and monitoring possible therapies for HD patients.
B G Jenkins; W J Koroshetz; M F Beal; B R Rosen
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Neurology     Volume:  43     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1993 Dec 
Date Detail:
Created Date:  1994-01-13     Completed Date:  1994-01-13     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2689-95     Citation Subset:  AIM; IM    
MGH-NMR Center, Massachusetts General Hospital, Boston.
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MeSH Terms
Aspartic Acid / analogs & derivatives,  metabolism
Basal Ganglia / metabolism*
Choline / metabolism
Creatine / metabolism
Energy Metabolism*
Huntington Disease / metabolism*
Lactates / metabolism
Lactic Acid
Magnetic Resonance Spectroscopy*
Middle Aged
Occipital Lobe / metabolism*
Reference Values
Grant Support
Reg. No./Substance:
0/Lactates; 0/Protons; 50-21-5/Lactic Acid; 56-84-8/Aspartic Acid; 57-00-1/Creatine; 62-49-7/Choline; 997-55-7/N-acetylaspartate

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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