Oey N A, van Vlies N, Wijburg F A, Wanders R J A, Attie-Bitach T, Vaz F M
Department of Pediatrics, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Placenta. 2006 Aug;27(8):841-6. doi: 10.1016/j.placenta.2005.10.002. Epub 2005 Nov 18.
Carnitine plays an indispensable role in fatty acid oxidation. Previous studies revealed that fetal carnitine is derived from the mother via transplacental transfer. Recent studies demonstrated the presence and importance of an active fatty acid oxidation system in the human placenta and in the human fetus. In view of these findings we decided to study carnitine metabolism in the fetal-placental unit by measuring carnitine metabolites, intermediary metabolites of carnitine biosynthesis, as well as the activity of carnitine biosynthesis enzymes in human term placenta, cord blood and selected embryonic and fetal tissues (5-20 weeks of development). Placenta contained low but detectable activity of gamma-butyrobetaine dioxygenase. This enzyme, which was considered to be expressed only in kidney, liver and brain, catalyzes the last step in the carnitine biosynthesis pathway. In addition, our results show that human fetal kidney, liver and spinal cord already have the capacity to synthesize carnitine. The ability of the placenta and fetus to synthesize carnitine suggests that in circumstances when maternal carnitine supply is limited, carnitine biosynthesis by the fetal-placental unit may supply sufficient carnitine for placental and fetal metabolism.
肉碱在脂肪酸氧化过程中发挥着不可或缺的作用。先前的研究表明,胎儿的肉碱是通过胎盘转运从母体获得的。近期研究证实了人体胎盘和胎儿中存在活跃的脂肪酸氧化系统及其重要性。鉴于这些发现,我们决定通过测量肉碱代谢产物、肉碱生物合成的中间代谢产物,以及足月胎盘、脐带血和选定的胚胎及胎儿组织(发育5至20周)中肉碱生物合成酶的活性,来研究胎儿 - 胎盘单位中的肉碱代谢。胎盘中γ-丁基甜菜碱双加氧酶的活性较低但可检测到。这种酶以前被认为仅在肾脏、肝脏和大脑中表达,它催化肉碱生物合成途径的最后一步。此外,我们的结果表明,人类胎儿的肾脏、肝脏和脊髓已经具备合成肉碱的能力。胎盘和胎儿合成肉碱的能力表明,在母体肉碱供应受限的情况下,胎儿 - 胎盘单位的肉碱生物合成可能为胎盘和胎儿的代谢提供足够的肉碱。