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Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.

Abstract:

Gangliosides are found at high levels in neuronal tissues where they play a variety of important functions. In the gangliosidoses, gangliosides accumulate because of defective activity of the lysosomal proteins responsible for their degradation, usually resulting in a rapidly progressive neurodegenerative disease. However, the molecular mechanism(s) leading from ganglioside accumulation to neurodegeneration is not known. We now examine the effect of ganglioside GM2 accumulation in a mouse mod...

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Publication status:
Published

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Publisher copy:
10.1074/jbc.M302964200

Authors


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Institution:
University of Oxford
Division:
MSD
Department:
Pharmacology
Role:
Author
Journal:
Journal of biological chemistry More from this journal
Volume:
278
Issue:
32
Pages:
29496-29501
Publication date:
2003-08-01
DOI:
EISSN:
1083-351X
ISSN:
0021-9258

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