AJCN 19th International Congress of Nutrition
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American Journal of Clinical Nutrition, Vol 9, 63-67, Copyright © 1961 by The American Society for Clinical Nutrition, Inc.

Sphingomyelin Synthesis in Niemann-Pick Disease

ALLEN C. CROCKER M.D.1 and VIVIAN B. MAYS B.S.1

1 From the Children's Cancer Research Foundation, The Children's Medical Center and the Department of Pathology, The Children's Hospital and Harvard Medical School, Boston, Massachusetts

A study has been made to investigate the rate at which synthesis of sphingomyelin utilizes tracer inorganic phosphate, with particular attention to the comparison with the whole phospholipid area. In vivo rates (twenty-four and forty-eight hours, rats) show sphingomyelin to be somewhat more slowly synthesized, but the ultimate specific activity approaches that of the total phospholipids. With tissue slices, followed for four hours, sphingomyelin production appears to be proceeding at an average of one half to two thirds the rate for the total phospholipids. Liver and spleen slices (and white blood cell suspensions) from patients with Niemann-Pick disease have been tested for in vitro lipid synthesis, but the reporting of results is handicapped by the presence of a large pool of previously formed lipid. Specific activity measurements (expressed in terms of unit weight of tissue) do not show evidence for increased synthesis of sphingomyelin in Niemann-Pick disease.






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