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.
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 neurodegene...
Main Authors: | Pelled, D, Lloyd-Evans, E, Riebeling, C, Jeyakumar, M, Platt, F, Futerman, A |
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Format: | Journal article |
Language: | English |
Published: |
2003
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