Lysosomal enzymes in normal and Chediak-Higashi blood leukocytes

Abstract

A method of blood granylocyte concentration and isolation of granules from both normal and neutropenic Chediak-Higashi syndrome (CHS) patients is described. the intracellular distribution of activity for several hydrolases in CHS granulocytes differs from normal; significantly more activity is present in the cytoplasmic fraction and correspondingly less is granule-associated. Isolated CHS granules are not more sensitive to the labilizing agents vitamin A, progesterone, or etiocholanolone. Specific activities of myeloperoxidase and ss-glucuronidase in CHS granulocytes are lower than normal while alkaline phosphatase is elevated. Other lysosomal enzyme activities are normal. Lysosomal enzyme distribution and content are similar in CHS and normal mononuclear cells. The possible significance of these findings is discussed.