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Clinical Chemistry, Vol 20, 444-446, Copyright © 1974 by the American Association for Clinical Chemistry
1 Department of Biochemistry and Biological Sciences Research Center, Child Development Institute, School of Medicine,
University of North Carolina, Chapel Hill, N. C. 27514.
We describe a gas-chromatographic procedure for determining methylmalonic acid in serum and urine. The sample is acidified and the compound is extracted from it with diethyl ether, derivatized with N,O-bis(trimethylsilyl)trifluoroacetamide, and gas chromatographed. Precision and sensitivity of the method were verified by recovery studies and by comparing the results with those for an established procedure in which ion-exchange column chromatography and colorimetry are used. We analyzed biological fluids from normal individuals and from a patient with methylmalonic aciduria to show that the procedure can be applied to diagnosis of disorders that result in abnormally large amounts of methylmalonic acid in urine and serum.
Submitted on December 20, 1973
Accepted on January 22, 1974
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