lysosomal enzymes


There are many differet enzymes in lysosomes. However they all function best in an acidic pH (5.0). In the table below some of the enzymes and catagories of enzyme are listed.

 enzymes

normal substrate

disease of

DNase and RNase

RNA and DNA

 

many different acid proteases

proteins

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polysaccharide and mucopolysaccharide degrading enzymes 

disaccharides, polysaccharides, glycolipids, glycoproteins, mucopolysaccharides

 Krabbe's disease (beta galactosidase deficient)

Pompe's diseaase (alpha glucosidase deficient)

Wollman's disease (acid lipase deficient)

 lipid degrading enzymes

 fatty acid esters, phospholipids

 Tay-Sach's disease (hexoseaminidase A deficient)

Farber's disease (ceramidase deficient)