Ganglioside GM2 catabolism is inhibited by storage compounds of mucopolysaccharidoses and by cationic amphiphilic drugs
S Anheuser, B Breiden, K Sandhoff - Molecular genetics and metabolism, 2019 - Elsevier
The catabolism of ganglioside GM2 is dependent on the lysosomal enzyme β-
hexosaminidase A and a supporting lipid transfer protein, the GM2 activator protein. A
genetically based disturbance of GM2 catabolism, leads to several subtypes of the GM2
gangliosidosis: Tay-Sachs disease, Sandhoff disease, the AB-variant and the B1-variant, all
of them having GM2 as major lysosomal storage compound. Further on it is known that the
gangliosides GM2 and GM3 accumulate as secondary storage compounds in …
hexosaminidase A and a supporting lipid transfer protein, the GM2 activator protein. A
genetically based disturbance of GM2 catabolism, leads to several subtypes of the GM2
gangliosidosis: Tay-Sachs disease, Sandhoff disease, the AB-variant and the B1-variant, all
of them having GM2 as major lysosomal storage compound. Further on it is known that the
gangliosides GM2 and GM3 accumulate as secondary storage compounds in …