Reactivating factors of human alpha-mannosidase mutant.
In a previous study, we compared the alpha-mannosidase from mannosidosis tissues to that from normal one and we characterized the mutant. In this work, we show that the mutant inactivation by dialysis is reversible in different conditions and we investigate the nature of mannosidosis reactivating factors. The results obtained on pathological tissue by dialysis and by addition of dialysis fluid (DF), pronase treated DF, amino acid mixture, bivalent ions: Ca++, Zn++, Mg++, Co++ DF containing EDTA or DF heated to 600 degrees C suggest the reactivating factor includes both peptides and ions.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000235:genetics / Q000378:metabolism*; D002412:Cations / Q000032:analysis; D055598:Chemical Phenomena; D002621:Chemistry; D003956:Dialysis; D004492:Edetic Acid / Q000032:analysis; D004793:Enzyme Reactivators / Q000032:analysis*; D005260:Female; D005333:Fetus / Q000378:metabolism; D006801:Humans; D008099:Liver / Q000201:enzymology; D008358:Mannose / Q000378:metabolism*; D008361:Mannosidases / Q000172:deficiency*; D009154:Mutation; D011247:Pregnancy; D043323:alpha-Mannosidase
D002412:Cations; D004793:Enzyme Reactivators; D004492:Edetic Acid; D008361:Mannosidases; D043323:alpha-Mannosidase; D008358:Mannose