In Lysosomal storage disorders, true is
Lysosomal storage diseases (LSDs); are a group of approximately 50 rare inherited metabolic disorders that result from defects in lysosomal function. Lysosomes are sacs of enzymes within cells that digest large molecules and pass the fragments on to other pas of the cell for recycling. This process requires several critical enzymes. If one of these enzymes is defective, because of a mutation, the large molecules accumulate within the cell, eventually killing it.
The lyososomes are deficient in the enzyme hydrolase
There is a defect in the fusion of lysosomes and phagosomes
There is a defect in the lysosomal membrane
There is increased degradation of heteroglycans
Metabolism of carbohydrate