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White Blood Cell Acid Hydrolases in Leukaemias, Mucopolysaccharidoses and Mannosidosis
Author(s) -
Hultberg B.,
Autio S.,
Berg B.,
ÖCkerman P. A.
Publication year - 1973
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1973.tb00071.x
Subject(s) - enzyme , isoelectric focusing , chemistry , enzyme replacement therapy , biochemistry , galactosidases , mucopolysaccharidosis i , mannosidase , mucolipidosis , medicine , beta galactosidase , disease , gene expression , gene
Simple, synthetic substrates, commercially available, were used for assay of five acid hydrolases (β‐galactosidase, β‐glucosidase, α‐mannosidase, N‐acetyl‐β‐glucosaminidase, and β‐glucuronidase) in human white blood cells from 23 controls and 25 patients with inborn storage disorders or leukaemia. Conditions for release of the enzyme activities, pH‐dependency, and properties on gel filtration and isoelectric focusing are described. In chronic myelocytic leukaemia high activities of α‐mannosidase were found, whereas the activities of all enzymes were low in chronic lymphocytic leukaemia. In acute myeloblast leukaemia enzyme activities varied. In two cases of mannosidosis very low values of α‐mannosidase were found.