Mock D M, Johnson S B, Holman R T
Department of Pediatrics, University of Iowa, Iowa City 52242.
J Nutr. 1988 Mar;118(3):342-8. doi: 10.1093/jn/118.3.342.
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 omega 6, omega 3 and omega 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.
在三名接受肠外营养期间出现生物素缺乏的患者中,测量了四种主要脂质类别(磷脂、胆固醇酯、甘油三酯和游离脂肪酸)的血清脂肪酸组成。相对于正常范围,每类脂质中奇数链脂肪酸(15:0或17:0或两者)的百分比组成均增加,并且这些异常情况在生物素治疗后通常恢复正常或向正常水平降低。还发现了ω6、ω3和ω9途径中特定脂肪酸的异常,但这些异常情况在生物素治疗后并未得到解决。这些数据为以下结论提供了证据,即生物素缺乏会导致人体中某些奇数链脂肪酸的组成增加,其机制可能与生物素依赖性酶丙酰辅酶A羧化酶先天性缺乏导致奇数链脂肪酸血症的机制相同。