Immunological properties of N-acetyl-beta-D-glucosaminidase of normal human liver and of GM2-gangliosidosis liver.
Antisera were raised to a partially purified preparation of human liver hexosaminidase and to highly purified preparations of hexosaminidase isoenzymes A and B. All the antisera precipitated the enzyme in an enzymically active form, which could be located on immunodiffusion and immunoelectrophoretic gels by using a histochemical substrate. The antisera to the purified isoenzymes were shown to react with hexosaminidase from human liver, kidney, brain and spleen, but did not cross-react with human liver beta-glucosidase, beta-galactosidase, alpha-mannosidase, beta-xylosidase, arylsulphatase or acid phosphatase. Hexosaminidases A and B were immunologically identical. The immunological properties of the hexosaminidases from livers of patients with three types of GM(2)-gangliosidoses were closely similar. No evidence could be found for cross-reacting material in enzyme-deficient states.
D000081:Acetamides; D000818:Animals; D002848:Chromatography, DEAE-Cellulose; D002850:Chromatography, Gel; D003429:Cross Reactions; D004591:Electrophoresis, Polyacrylamide Gel; D005732:Gangliosides; D006596:Hexosaminidases / Q000302:isolation & purification / Q000378:metabolism*; D006801:Humans; D007106:Immune Sera; D005779:Immunodiffusion; D007122:Immunoelectrophoresis; D007525:Isoelectric Focusing; D007527:Isoenzymes / Q000378:metabolism; D008052:Lipid Metabolism, Inborn Errors / Q000201:enzymology*; D008099:Liver / Q000201:enzymology*; D008832:Microchemistry; D011817:Rabbits / Q000276:immunology; D013050:Spectrometry, Fluorescence
D000081:Acetamides; D005732:Gangliosides; D007106:Immune Sera; D007527:Isoenzymes; D006596:Hexosaminidases