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Department of Pediatrics, Division of Gastroenterology and Program in Human Nutrition, University of Iowa, Iowa City, IA 52242 * The Hormel Institute, University of Minnesota, Austin, MN 55912
In three patients who developed biotin deficiency during parenteral alimentation, serum fatty acid compositions of the four major lipid classes (phospholipid, cholesteryl ester, triglyceride and free fatty acid) were measured. Relative to the normal range, percent compositions of an odd-chain fatty acid (either 15:0 or 17:0 or both) were increased in each class of lipid, and these abnormalities generally returned to normal or decreased toward normal with biotin therapy. Abnormalities in particular fatty acids in the
6,
3 and
9 pathways were also found, but these abnormalities did not resolve with biotin therapy. These data provide evidence in favor of the conclusion that biotin deficiency causes increases in the composition of some odd-chain fatty acids in humans, perhaps by the same mechanism that leads to odd-chain fatty acidemia in the inborn deficiency of the biotin-dependent enzyme propionyl-CoA carboxylase.
KEY WORDS: biotin deficiency odd-chain fatty acids polyunsaturated fatty acids
1 Poster presentation, Federation of American Societies for Experimental Biology 71st Annual Meeting, Washington, DC, March 1987 [Mock, N., Mock, D., Holman, R. & Johnson, S. (1987) Effects of biotin deficiency on fatty acid (FA) composition of human and the rat. Fed. Proc. 46: 1489 (abs.)].
2 This study was supported by Grant No. RO1-DK36823 from the National Institute for Diabetes and Digestive and Kidney Diseases of the National Institutes of Health (D.M.), and the Hormel Foundation.
3 Recipient of Research Career Development Award No. KO4 DK01810 from the Institute of Diabetes and Digestive and Kidney Diseases of the National Institutes of Health.
4 To whom reprint requests should be addressed.
Manuscript received 22 June 1987. Revision accepted 6 November 1987.