Lysosomal glycosidase deficiencies

Deficiencies of lysosomal glycosidases cause partially degraded carbohydrates from proteoglycans, glycoproteins, and glycolipids to accumulate within membrane-enclosed vesicles inside cells. These residual bodies can cause marked enlargement of the organ with impairment of its function. 

Several lysosomal storage diseases result from defective expression of specific glycosidases. The intracellular accumulation of unprocessed carbohydrate substrates leads to aberrant cellular structure and cell death. Thus, deficient or defective hexosaminidase results in either Tay-Sachs or Sandhoff disease [157]. Fucosidosis, another neurovisceral storage disease, is caused by defective a-L-fucosidase expression. 

A patient was diagnosed with a deficiency of the lysosomal enzyme a-glycosidase. The name of the deficient enzyme suggests that it hydrolyzes a glycosidic bond, which is a bond formed... 

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