Subcellular localization of acid car☐ypeptidase in rat liver and human fibroblasts

The subcellular distribution of acid car☐ypeptidase was investigated in rat liver, normal human skin (CRL 1501) and lung (WI-38) fibroblasts, galactosialidosis skin fibroblasts (GM 00806) and transformed lung fibroblasts (WI-38 VA 13). Results of differential and isopycnic centrifugations and osmotic activation experiments clearly indicate that the enzyme is located in lysosomes, in agreement with observations suggesting that car☐ypeptidase is the protective protein of the ‘Galjaard complex’ which is defective in galactosialidosis.