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Journal of Lipid Research, Vol. 14, 69-77, January 1973
Copyright © 1973 by Lipid Research, Inc.

Branched-chain and odd-numbered fatty acids and aldehydes in the nervous system of a patient with deranged vitamin B₁₂ metabolism

Yasuo Kishimoto , Marcia Williams , Hugo W. Moser , Charles Hignite , and Klaus Biemann

Eunice Kennedy Shriver Center for Mental Retardation, Waltham, Massachusetts 02154; Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts 02114; and Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139

A mixture of isomers of methylhexadecanoic acid was isolated from glycerolipids of brain, spinal cord, and sciatic nerve of a patient who died from methylmalonic aciduria, a disease in which vitamin B₁₂ is not converted to deoxyadenosyl B₁₂. The isomers were identified by gas-liquid chromatographic-mass spectrometric analyses, and the data indicated that the points of methyl branching are located predominantly on the even-numbered carbon atoms. The concentration of these branched-chain acids among the glycerolipid fatty acids in the patient's nervous system was at least 0.3-0.9%, while the control tissues contained no more than a trace amount, if any, of these acids. In the spinal cord, these branched acids were distributed among all phosphatides and were in highest concentration on the ßbeta; position of phosphatidylcholine. On the other hand, most extraneural tissues contained these acids in much lower concentrations; there were only trace amounts in liver, kidney, muscle, and skin, and 0.2, 0.2, and 0.5% in total ester-linked fatty acids in spleen, duodenum, and lung, respectively. A second abnormality was the 6-13-fold increase in 15:0 and 17:0 fatty acids in all of the glycerolipids in the nervous system of the patient. This abnormality was also observed to a somewhat smaller extent in every extraneural tissue examined. The C₁₇ aldehydes of phosphatidylethanolamine plasmalogens from the spinal cord of the patient were identified by converting them to the corresponding dimethylacetals. 17:0 dimethylacetal accounted for nearly 10% of total dimethylacetals. There were no abnormalities in total lipids, cholesterol, cerebrosides, and sphingomyelins.

Supplementary key words gas-liquid chromatography-mass spectrometry • dimethylacetal • methylmalonic aciduria • brain • spinal cord • sciatic nerve • extraneural tissues

Submitted on May 24, 1972
Accepted on September 15, 1972

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