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Clinical Chemistry 21: 725-729, 1975;
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Clinical Chemistry, Vol 21, 725-729, Copyright © 1975 by the American Association for Clinical Chemistry

Above-Normal Urinary Excretion of Urinary Ceramides in Farber's Disease, and Characterization of Their Components by High-Performance Liquid Chromatography

Masao Iwamori 1 and Hugo W. Moser 1

1 Eunice Kennedy Shriver Center for Mental Retardation at the Walter B. Fernald State School, Waltham, Mass. 02154; and Department of Neurology, Massachusetts General Hospital, Boston, Mass. 02114.

We compared the sphingolipid content of urine from a patient with Farber's disease with that of control urine. The ceramides were measured by high-performance liquid chromatography. The patient's urine contained 1.2 µg of ceramides per milligram of creatinine, more than 200-fold the normal amount. The urinary ceramides were isolated by high-performance liquid chromatography for further identification. They contained mainly nonhydroxy fatty acids and only a small quantity of those with 2-hydroxy fatty acids. This contrasts with the previously described composition of the patient's renal and cerebellar tissue. The fatty acid and long-chain base compositions of the urinary ceramides containing nonhydroxy fatty acids were nearly identical to those of the patient's kidney.


Key Words: metachromatic leukodystrophy • sphlngolipids • lipogranulomatosis • sphingolipidosis • results for tissue compared • normal values

Submitted on December 6, 1974
Accepted on February 21, 1975






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Copyright © 1975 by the American Association for Clinical Chemistry.