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The core-specific lysosomal alpha(1-6)-mannosidase activity depends on aspartamidohydrolase activity
1Unité Mixte de Recherche du CNRS no. 111, Université des Sciences et Technologies de Lille, Villeneuve d'Ascq, France.
The Biochemical Journal
|February 1, 1994
Abstract:
The substrate specificity of the core-specific rat liver lysosomal alpha(1-6)-mannosidase was investigated using mannosylated oligosaccharides and glycoasparagines. Hydrolysis of Man(alpha 1-6) linkage hydrolysis was demonstrated to follow the action of endoglycosidases, namely aspartyl-N-acetyl-beta-D-glucosaminidase and endo-N-acetyl-beta-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.