CORE-SPECIFIC LYSOSOMAL ALPHA(1-6)-MANNOSIDASE ACTIVITY DEPENDS ON ASPARTAMIDOHYDROLASE ACTIVITY

The substrate specificity of the core-specific rat liver lysosomal alp ha(1-6)-mannosidase was investigated using mannosylated oligosaccharid es and glycoasparagines. Hydrolysis of Man(alpha 1-6) linkage hydrolys is was demonstrated to follow the action of endoglycosidases, namely a spartyl-N-acetyl-beta-D-glucosaminidase and endo-N-acetyl-beta-D-gluco saminidase. The results are discussed with respect to the nature of th e carbohydrate materials stored in the tissues and excreted in the uri ne from patients suffering from aspartylglucosaminuria and fucosidosis .