Above-Normal Urinary Excretion of Urinary Ceramides in Farber's Disease, and Characterization of Their Components by High-Performance Liquid Chromatography
Open Access
- 1 May 1975
- journal article
- Published by Oxford University Press (OUP) in Clinical Chemistry
- Vol. 21 (6) , 725-729
- https://doi.org/10.1093/clinchem/21.6.725
Abstract
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.Keywords
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