Properties of α-Mannosidase in Mannosidosis

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.

Key Words:

• Liver
• lysosomes
• α-mannosidase
• mannosidosis