caa a22 4500 467932506 CHVBK 20180406152947.0 cr unu---uuuuu 170328e20060201xx s 000 0 eng 10.1007/s10545-006-0199-x doi (NATIONALLICENCE)springer-10.1007/s10545-006-0199-x Fatty acid oxidation in the human fetus: Implications for fetal and adult disease [Elektronische Daten] [Nadia Oey, Jos Ruiter, Tania Attié-Bitach, Lodewijk IJlst, Ronald Wanders, Frits Wijburg] Summary: Studies in the last few years have shown a remarkably high activity of fatty acid oxidation (FAO) enzymes in human placenta. We have recently shown mRNA expression as well as enzymatic activity of long-chain FAO enzymes in the human embryo and fetus. In this study we show activity of the FAO enzymes carnitine palmitoyltranferase 1, medium-chain acyl-CoA dehydrogenase and short-chain hydroxyacyl-CoA dehydrogenase in embryonic and fetal tissues. In addition, we show the presence of different acylcarnitines in fetal liver and kidney, which substantiates the notion that the mitochondrial FAO enzymes are not only present in human fetal tissues but also metabolically active. In a glucose-rich environment FAO might be necessary for additional ATP production from fatty acids, but also for the breakdown of fatty acids that are products of the turnover of membranes in the growing fetus. The importance of FAO in the human embryo and fetus is further stressed by the fact that a higher frequency of prematurity, intrauterine growth retardation, fetal morbidity and intrauterine death is noted in long-chain FAO defects. Furthermore, in animal studies, gestational loss during early embryonic development has been observed as a consequence of disturbed FAO. Finally, there are indications that regulation of activity of FAO during fetal development might not only be important for fetal life but may also have implications for health and disease in adulthood. SSIEM and Springer, 2006 Oey Nadia Department of Pediatrics (G8-205), Emma Children's Hospital AMC, Academic Medical Centre, PO Box 22660, NL-1100 DD, Amsterdam, The Netherlands aut Ruiter Jos Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Attié-Bitach Tania Département de Génetique et Unité INSERM U-393, Hôpital Necker-Enfants Malades, Paris Cedex 15, France aut IJlst Lodewijk Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut Wanders Ronald Department of Pediatrics (G8-205), Emma Children's Hospital AMC, Academic Medical Centre, PO Box 22660, NL-1100 DD, Amsterdam, The Netherlands aut Wijburg Frits Department of Pediatrics (G8-205), Emma Children's Hospital AMC, Academic Medical Centre, PO Box 22660, NL-1100 DD, Amsterdam, The Netherlands aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/1(2006-02-01), 71-75 0141-8955 29:1<71 2006 29 10545 https://doi.org/10.1007/s10545-006-0199-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0199-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Oey Nadia Department of Pediatrics (G8-205), Emma Children's Hospital AMC, Academic Medical Centre, PO Box 22660, NL-1100 DD, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Ruiter Jos Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Attié-Bitach Tania Département de Génetique et Unité INSERM U-393, Hôpital Necker-Enfants Malades, Paris Cedex 15, France aut NATIONALLICENCE 700 1- IJlst Lodewijk Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Wanders Ronald Department of Pediatrics (G8-205), Emma Children's Hospital AMC, Academic Medical Centre, PO Box 22660, NL-1100 DD, Amsterdam, The Netherlands aut NATIONALLICENCE 700 1- Wijburg Frits Department of Pediatrics (G8-205), Emma Children's Hospital AMC, Academic Medical Centre, PO Box 22660, NL-1100 DD, Amsterdam, The Netherlands aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/1(2006-02-01), 71-75 0141-8955 29:1<71 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer