Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activity[S]

Ganglioside GM2 is the major lysosomal storage compound of Tay-Sachs disease. It also accumulates in Niemann-Pick disease types A and B with primary storage of SM and with cholesterol in type C. Reconstitution of GM2 catabolism with β-hexosaminidase A and GM2 activator protein (GM2AP) at uncharged l...

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Bibliographic Details
Main Authors: Susi Anheuser, Bernadette Breiden, Günter Schwarzmann, Konrad Sandhoff
Format: Article
Language:English
Published: Elsevier 2015-09-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520355048