Lysosomal enzymes glycoprotein degradation

The oligosaccharidoses are a group of lysosomal storage disorders characterized by defects of glycoprotein degradation due to the deficiency of specific lysosomal enzymes (Fig. 19.1). All are inherited as autosomal recessive traits. 

Apart from the amidasc and muramidase listed in Table VIII, the other lysosomal enzymes have acid pH optima, so that these hydrolases may well be unsusceptible to mutual attack until the pH of the medium becomes acid (see Shamberger, 1969). That the glycoprotein hydrolases might be resistant to degradation by lys(jsomal cathepsins and glycosidases would seem likely, but speculation on the basis for any such resistance would be unprofitable at present. 

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