The tissue distribution of hexosaminidase S and hexosaminidase C.
The proportion of hex S to hex C in normal and Sandhoff's fibroblasts was determined to be between 1:1 and 1:2 by differential staining of hex S at pH 4.4 with 4-methylumbelliferyl-beta-N-acetylgalactosaminide and of hex C at pH 7.0 with 4-methylumbelliferyl-beta-N-acetylglucosaminide. Hex S and hex C were also semi-quantitated in various normal tissues--brain, liver, spleen, heart, kidney, intestine, placenta, skeletal muscle and fibroblasts. Hex C was most prominent in brain and, somewhat less so, in liver, skeletal muscle and fibroblasts. The greatest amount of hex S activity was found in fibroblast, but it was also observed in lesser amounts in liver, kidney, intestine and placenta.
D004586:Electrophoresis; D005347:Fibroblasts / Q000201:enzymology; D005733:Gangliosidoses / Q000201:enzymology*; D006596:Hexosaminidases / Q000378:metabolism*; D006801:Humans; D014018:Tissue Distribution
D006596:Hexosaminidases