title: Effect of neuraminidase on the chromatographic behaviour of eleven acid hydrolases from human liver and plasma.
abstract: 1. The elution profiles of eleven acid hydrolases from human liver and plasma were directly compared using a system whereby a single salt gradient was simultaneously applied to two DEAE-cellulose chromatographic columns. 2. Plasma alpha-L-fucosidase, alpha-mannosidase, alpha-galactosidase and alpha-glucosidase isoenzymes were eluted at higher salt concentrations than the corresponding liver isoenzymes whereasbeta-N-acetylglucosaminidase, beta-galactosidase, beta-glucosidase, exo-1,4-beta-xylosidase and alpha-L-arabinofuranosidase isoenzymes were eluted at lower salt concentrations. The elution profiles of beta-glucuronidase and acid phosphatase weremore complex. 3. After incubation with neuraminidase most plasma hydrolases were eluted at lower salt concentrations, however the elution patterns of beta-glucosidase, beta-xylosidase and acid phosphatase were not altered. 4. Preincubation with neuraminidase had no effect on the elution profiles of six liver hydrolases whereas the major isoenzymes of alpha-mannosidase, beta-galactosidase and alpha-L-arabinofuranosidase were eluted at markedly lower salt concentrations. Liver alpha-fucosidase and alpha-galactosidase were eluted at slightly lower salt concentrations afterincubation with neuraminidase. 5. The results are discussed in relation to thepathogenesis of Mucolipidosis II (I-cell disease), and the synthesis and packaging of lysosomal enzymes.
mesh_terms: D000118:Acetylglucosaminidase / Q000378:metabolism; D001089:Arabinose; D002848:Chromatography, DEAE-Cellulose; D003016:Clostridium perfringens / Q000201:enzymology; D005696:Galactosidases / Q000378:metabolism; D005959:Glucosidases / Q000378:metabolism; D006026:Glycoside Hydrolases / Q000097:blood / Q000302:isolation & purification / Q000378:metabolism*; D006801:Humans; D007527:Isoenzymes / Q000378:metabolism; D008099:Liver / Q000201:enzymology*; D009439:Neuraminidase / Q000494:pharmacology*; D014994:Xylose; D005644:alpha-L-Fucosidase / Q000378:metabolism
chemical_list: D007527:Isoenzymes; D014994:Xylose; D001089:Arabinose; D005696:Galactosidases; D005959:Glucosidases; D006026:Glycoside Hydrolases; D009439:Neuraminidase; D005644:alpha-L-Fucosidase; D000118:Acetylglucosaminidase