The biochemical basis of gangliosidoses.

Brain/enzymology Child Fibroblasts/enzymology G(M1) Ganglioside/metabolism G(M2) Activator Protein G(M2) Ganglioside/metabolism Gangliosides/metabolism Gangliosidoses/enzymology,genetics Glycoproteins Hexosaminidases/deficiency Humans Lysosomes/enzymology Mutation Protein Deficiency Proteins Sandhoff Disease/enzymology,genetics Saposins Sphingolipid Activator Proteins Tay-Sachs Disease/enzymology,genetics beta-Galactosidase/deficiency beta-N-Acetylhexosaminidases

G(M2) Activator Protein Gangliosides Glycoproteins PSAP protein, human Proteins Saposins Sphingolipid Activator Proteins G(M2) Ganglioside G(M1) Ganglioside Hexosaminidases acid beta-galactosidase beta-Galactosidase beta-N-Acetylhexosaminidases