Alteration of Sphingolipid Metabolism in Leukocytes from Patients with the Chediak–Higashi Syndrome

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Abstract
The synthesis and degradation of sphingolipids by mixed peripheral leukocytes from four patients with the Chediak—Higashi syndrome were studied in vitro. Incorporation of glucose uniformly labeled with radioactive carbon into glucocerebroside and radioactive phosphate into sphingomyelin was greater than normal, as was the degradation of specifically labeled sphingomyelin and glucocerebroside. These observations can be interpreted as demonstrating an accelerated turnover of sphingolipids in leukocytes from patients with the Chediak—Higashi syndrome.