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The tissue distribution of hexosaminidase S and hexosaminidase C
Author(s) -
BEUTLEE ERNEST,
KUHL WANDA
Publication year - 1977
Publication title -
annals of human genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.537
H-Index - 77
eISSN - 1469-1809
pISSN - 0003-4800
DOI - 10.1111/j.1469-1809.1977.tb01911.x
Subject(s) - hexosaminidase , distribution (mathematics) , biology , chemistry , biochemistry , enzyme , mathematics , mathematical analysis
SUMMARY 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‐β‐ N ‐acetylgalactosaminide and of hex C at pH 7.0 with 4‐methylumbelliferyl‐β‐ 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.