title: Enzyme activities of the mitochondrial energy generating system in skeletal muscle tissue of preterm and fullterm neonates.
abstract: Quadriceps muscle specimens from autopsy of 28 neonates (gestational age 25-42 weeks) were investigated to determine pyruvate and malate oxidation rates and several enzymes of the mitochondrial oxidative process. In general, the levels of all mitochondrial parameters measured, including carnitine levels, were lower in the neonates who died within the first week of life than those in the control group (age > 5 years). Pyruvate and malate oxidation rates (P < 0.05), activities of pyruvate dehydrogenase complex (P < 0.10) and succinate: cytochrome c oxidoreductase (P < 0.05) increased significantly with gestational age. Pyruvate oxidation rates (P < 0.05) as well as activities of citrate synthase (P < 0.05) and NADH:Q1 oxidoreductase (P < 0.05) were significantly lower in the group of very preterm infants at an age of 1-7 days compared with very preterm infants at an age between 3-8 weeks. We conclude from our study that special reference values are necessary for a correct biochemical diagnosis of mitochondrial encephalomyopathies in the neonatal period. Differences between preterm and fullterm children of the same age (1 week) indicate a maturational process in human muscle tissue during gestation. Comparison of two different age groups within the very preterm neonates point to a postnatal maturation of the mitochondrial energy metabolism, at least in preterm neonates.
authors: Department of Paediatrics, University of Innsbruck, Austria.
authors: W
authors: W
authors: Sperl
authors: R C
authors: RC
authors: Sengers
authors: J M
authors: JM
authors: Trijbels
authors: W
authors: W
authors: Ruitenbeek
authors: W H
authors: WH
authors: Doesburg
authors: J A
authors: JA
authors: Smeitink
authors: L A
authors: LA
authors: Kollée
authors: J M
authors: JM
authors: Boon
mesh_terms: D002331:Carnitine / Q000032:analysis; D004734:Energy Metabolism / Q000502:physiology*; D005260:Female; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature / Q000378:metabolism*; D008293:Malates / Q000378:metabolism; D008297:Male; D008931:Mitochondria, Muscle / Q000201:enzymology*; D011768:Pyruvate Dehydrogenase Complex / Q000032:analysis; D011773:Pyruvates / Q000378:metabolism
chemical_list: D008293:Malates; D011768:Pyruvate Dehydrogenase Complex; D011773:Pyruvates; C030298:malic acid; D002331:Carnitine