Document Detail

Ganglioside alterations in the central and peripheral nervous systems of patients with Creutzfeldt-Jakob disease.
MedLine Citation:
PMID:  9117545     Owner:  NLM     Status:  MEDLINE    
We have examined the distribution and composition of gangliosides in central and peripheral nervous tissues from two patients with Creutzfeldt-Jakob disease (CJD). There were marked decreases in total ganglioside levels in CJD, with reductions in the cerebral cortex and cerebellum in the order of 20-30% and 50% of control values, respectively, though in spinal cord and sciatic nerve total gangliosides were not significantly altered. The percentage distribution of individual gangliosides was characterized by marked increases in GD3 and GD2, contrasting with severe decreases in GD1a, GD1b, GT1b and GQ1b; such changes were found throughout the patients' nervous tissues. An abnormal long-chain base composition was detected with the d20:1 component being decreased to less than 50% of control values, in the cerebrum, cerebellum and spinal cord. Changes in gangliosides occurred even in those tissues not severely affected neuropathologically. These ganglioside abnormalities are discussed in relationship to the pathogenesis of CJD.
Y Ohtani; Y Tamai; Y Ohnuki; S Miura
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurodegeneration : a journal for neurodegenerative disorders, neuroprotection, and neuroregeneration     Volume:  5     ISSN:  1055-8330     ISO Abbreviation:  Neurodegeneration     Publication Date:  1996 Dec 
Date Detail:
Created Date:  1997-04-24     Completed Date:  1997-04-24     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9209022     Medline TA:  Neurodegeneration     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  331-8     Citation Subset:  IM    
Department of Psychiatry, School of Medicine, Kitasato University, Kanagawa, Japan.
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MeSH Terms
Central Nervous System / metabolism*
Chromatography, Thin Layer
Creutzfeldt-Jakob Syndrome / metabolism*
Gangliosides / metabolism*
Middle Aged
Peripheral Nerves / metabolism*
Reference Values
Reg. No./Substance:

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