L-carnitine is synthesized in the human fetal-placental unit: potential roles in placental and fetal metabolism.
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.
Oey
N A
NA
Department of Pediatrics, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
van Vlies
N
N
Wijburg
F A
FA
Wanders
R J A
RJ
Attie-Bitach
T
T
Vaz
F M
FM
D002331:Carnitine / Q000032:analysis / Q000378:metabolism*; D005260:Female; D005333:Fetus / Q000737:chemistry / Q000201:enzymology / Q000378:metabolism*; D006801:Humans; D010920:Placenta / Q000737:chemistry / Q000201:enzymology / Q000378:metabolism*; D011247:Pregnancy / Q000378:metabolism*; D014018:Tissue Distribution; D050563:gamma-Butyrobetaine Dioxygenase / Q000032:analysis*
D050563:gamma-Butyrobetaine Dioxygenase; D002331:Carnitine