PMID- 20065299 OWN - NLM STAT- MEDLINE DCOM- 20100422 LR - 20151119 IS - 1933-7205 (Electronic) IS - 1933-7191 (Linking) VI - 17 IP - 3 DP - 2010 Mar TI - Preeclampsia and HELLP syndrome: impaired mitochondrial function in umbilical endothelial cells. PG - 219-26 LID - 10.1177/1933719109351597 [doi] AB - Preeclampsia (PE) and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome have been linked to congenital fetal disorders of mitochondrial fatty acid oxidation (FAO). Different incidences may argue for the association of noncongenital alterations of mitochondrial energy metabolism with PE/HELLP syndrome. We studied human umbilical vein endothelial cells [HUVEC] as selected part of the feto-placental unit from uncomplicated (n = 46) and diseased (n = 27; 17 PE and 10 HELLP) pregnancies by measuring the overall FAO, carnitine palmitoyltransferase 2 (CPT2), respiratory chain (RC) complexes I-V, citratesynthase (CS), lactatedehydrogenase (LDH), hexokinase (HK), phosphofructokinase (PFK), and energy rich phosphates. Maternal and infantile acylcarnitines in blood were investigated post partum. Overall FAO, RC complexes II-V, and CS were significantly compromised in HUVEC from complicated pregnancies; impairment of complexes I + III was not significant. CPT2 and energy charges were unaffected. Lactatedehydrogenase and PFK from complicated pregnancies were upregulated, and HK remained constant. In blood, carnitine was elevated in diseased women and their children, acylcarnitines were higher in affected infants. Impaired mitochondrial function in HUVEC is associated with PE/HELLP syndrome and may be involved in the pathophysiology of these diseases. FAU - Illsinger, Sabine AU - Illsinger S AD - Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany. illsinger.sabine@mh-hannover.de FAU - Janzen, Nils AU - Janzen N FAU - Sander, Stefanie AU - Sander S FAU - Schmidt, Karl-Heinz AU - Schmidt KH FAU - Bednarczyk, Jolanthe AU - Bednarczyk J FAU - Mallunat, Lisa AU - Mallunat L FAU - Bode, Julia AU - Bode J FAU - Hagebolling, Friederike AU - Hagebolling F FAU - Hoy, Ludwig AU - Hoy L FAU - Lucke, Thomas AU - Lucke T FAU - Hass, Ralf AU - Hass R FAU - Das, Anibh M AU - Das AM LA - eng PT - Journal Article DEP - 20100111 PL - United States TA - Reprod Sci JT - Reproductive sciences (Thousand Oaks, Calif.) JID - 101291249 RN - 0 (Fatty Acids) RN - 0 (Multienzyme Complexes) RN - 0 (acylcarnitine) RN - EC 1.1.1.27 (L-Lactate Dehydrogenase) RN - EC 2.3.1.21 (Carnitine O-Palmitoyltransferase) RN - EC 2.3.3.1 (Citrate (si)-Synthase) RN - EC 2.7.1 - (Phosphofructokinases) RN - S7UI8SM58A (Carnitine) SB - IM MH - Adult MH - Carnitine/analogs & derivatives/blood MH - Carnitine O-Palmitoyltransferase/metabolism MH - Citrate (si)-Synthase/metabolism MH - Electron Transport MH - Endothelial Cells/enzymology/*metabolism/ultrastructure MH - Energy Metabolism MH - Fatty Acids/metabolism MH - Female MH - Glycolysis MH - HELLP Syndrome/etiology/*metabolism MH - Humans MH - Infant, Newborn MH - L-Lactate Dehydrogenase/metabolism MH - Mitochondria/enzymology/*metabolism MH - Mitochondrial Diseases/complications/enzymology/*metabolism MH - Multienzyme Complexes/metabolism MH - Oxidation-Reduction MH - Phosphofructokinases/metabolism MH - Pre-Eclampsia/etiology/*metabolism MH - Pregnancy MH - *Umbilical Veins EDAT- 2010/01/13 06:00 MHDA- 2010/04/23 06:00 CRDT- 2010/01/13 06:00 PHST- 2010/01/13 06:00 [entrez] PHST- 2010/01/13 06:00 [pubmed] PHST- 2010/04/23 06:00 [medline] AID - 1933719109351597 [pii] AID - 10.1177/1933719109351597 [doi] PST - ppublish SO - Reprod Sci. 2010 Mar;17(3):219-26. doi: 10.1177/1933719109351597. Epub 2010 Jan 11.