Characterization and tissue distribution of N-acetyl hexosaminidase C: suggestive evidence for a separate hexosaminidase locus.
1. An electrophoretic system in which N-acetyl hexosaminidase C (HEX(C)) MIGRATES LESS ANODALLY THAN N-acetyl hexosaminidase A (HEX(A)) is described. 2. HEX(C) is shown to differ from HEX(A) and HEX(B) in substrate specificity, molecular size and affinity for Concanavalin-A. 3. HEX(C) is present in a wide range of adult and foetal tissues and in tissues from patients with Tay-Sachs and Sandhoff's diseases. It is particularly prominent in brain, testis, thymus and lymphoblastoid cell extracts and in several foetal tissues. 4. It is suggested that HEX(C) is coded at a separate gene locus from HEX(A) and HEX(B).
D000118:Acetylglucosaminidase / Q000032:analysis*; D000328:Adult; D001921:Brain / Q000201:enzymology; D002478:Cells, Cultured; D002648:Child; D002874:Chromosome Mapping; D003208:Concanavalin A; D004592:Electrophoresis, Starch Gel; D005333:Fetus / Q000201:enzymology; D005347:Fibroblasts / Q000201:enzymology; D006596:Hexosaminidases / Q000032:analysis*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007526:Isoelectric Point; D007527:Isoenzymes / Q000032:analysis*; D008064:Lipidoses / Q000201:enzymology / Q000235:genetics; D008221:Lymphoid Tissue / Q000201:enzymology; D008297:Male; D008970:Molecular Weight; D013737:Testis / Q000201:enzymology; D013950:Thymus Gland / Q000201:enzymology
D007527:Isoenzymes; D003208:Concanavalin A; D006596:Hexosaminidases; D000118:Acetylglucosaminidase