Properties of α-Mannosidase in Mannosidosis
Author:
- 1 January 1970
- journal article
- research article
- Published by Taylor & Francis in Scandinavian Journal of Clinical and Laboratory Investigation
- Vol. 26 (2) , 155-159
Abstract
The rest activity of α-tnannosidase in a patient with mannosidosis was investigated. By isoelectric focusing both patient and controls showed heterogeneity. In gel filtration experiments the rest activity of the patient had a lower apparent molecular weight compared to controls. Different pH optima were also seen. When the mannose-rich oligosaccharides accumulated by the patient were used as substrate for α-mannosidase, a reduced degrada tion could be shown in enzyme preparations from the patient.Keywords
This publication has 20 references indexed in Scilit:
- β-Glucosidase activities in human tissues findings in Gaucher's diseaseClinica Chimica Acta, 1970
- Gargoylism: hydrolysis of β-galactosides and tissue accumulation of galactose- and mannose-containing compoundsJournal of Clinical Investigation, 1970
- N-ACETYLHEXOSAMINIDASE ACTIVITIES IN TAY-SACHS DISEASEThe Lancet, 1969
- Mannosidosis: A clinical and histopathologic studyThe Journal of Pediatrics, 1969
- Degradation of polysaccharides, mucopolysaccharides, and glycoproteins by lysosomal glycosidasesArchives of Biochemistry and Biophysics, 1969
- Properties of 4-Methylumbelliferyl-β-Galactosidase Activities in Human LiverScandinavian Journal of Clinical and Laboratory Investigation, 1969
- Glycosidases in bovine milk: α-mannosidase and its inhibition by zwitterionsCanadian Journal of Biochemistry, 1968
- Characterization of beef-liver glycosidases possibly involved in glycoprotein degradationArchives of Biochemistry and Biophysics, 1968
- Action of beef-liver enzymes on a glycoprotein substrateArchives of Biochemistry and Biophysics, 1968
- Mammalian glycosidases. 2. Properties of α-mannosidase and β-galactosidase from rat epididymisBiochemical Journal, 1959