Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice.
Glycosphingolipid storage diseases are a group of inherited metabolic diseases in which glycosphingolipids accumulate due to their impaired lysosomal breakdown. Splenic B cells isolated from NPC1, Sandhoff, GM1-gangliosidosis and Fabry disease mouse models showed large (20- to 30-fold) increases in...
Main Authors: | te Vruchte, D, Jeans, A, Platt, F, Sillence, D |
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Format: | Journal article |
Language: | English |
Published: |
2010
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