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...

Descripció completa

Dades bibliogràfiques
Autors principals: te Vruchte, D, Jeans, A, Platt, F, Sillence, D
Format: Journal article
Idioma:English
Publicat: 2010