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.
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Björn Hultberg (1970) studied this question.
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