The core-specific lysosomal α(1-6)-mannosidase activity depends on aspartamidohydrolase activity

Abstract

The substrate specificity of the core-specific rat liver lysosomal α(1-6)-mannosidase was investigated using mannosylated oligosaccharides and glycoasparagines. Hydrolysis of Man(α1-6) linkage hydrolysis was demonstrated to follow the action of endoglycosidases, namely aspartyl-N-acetyl-β-D-glucosaminidase and endo-acetyl-β-D-glucosaminidase. The results are discussed with respect to the nature of the carbohydrate materials stored in the tissues and excreted in the urine from patients suffering from aspartylglucosaminuria and fucosidosis.