BACKGROUND AND AIMS: Most diseases in premature neonates are secondary to immaturity of various organ systems. Also the inadequate capacity of mitochondrial energy production may play an important role in the neonatal morbidity. SUBJECTS AND METHODS: The activities and amount of respiratory chain (RC) complexes, pyruvate dehydrogenase (PDH) and citrate synthase (CS) were analysed in isolated muscle mitochondria obtained at autopsy in 19 premature neonates using spectrophotometric and radioenzymatic methods and blue-native electrophoresis and Western blotting. Two groups of children recommended for muscle biopsy at the age of 0.5-2 and 3-18 years served as controls. RESULTS: In premature neonates, the activities of RC complexes III, IV, PDH and CS were markedly lower in comparison with older children. On the contrary, the activity of complex I was higher in premature neonates than in older children. The ratios between RC complexes I, II and III and CS were significantly higher in premature neonates in comparison with older children. In addition, the protein amount of RC complexes and PDH subunits were lower in premature neonates in comparison with older children. CONCLUSION: The results of our study document the age-dependent differences in activities of PDH and respiratory chain complexes in early childhood. Lower functional capacity of mitochondrial energy-providing system in critically ill neonates may be explained by combination of various factors including the delay in maturation of PDH and respiratory chain complexes in very premature neonates and increased degradation of mitochondrial proteins in connection with sepsis, tissue hypoperfusion or hypoxemia.

• MeSH
• citrátsynthasa metabolismus   MeSH
• dítě MeSH
• kojenec MeSH
• kosterní svaly cytologie   enzymologie   fyziologie   MeSH
• lidé MeSH
• mladiství MeSH
• následné studie MeSH
• novorozenec nedonošený fyziologie   metabolismus   MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• pyruvátdehydrogenasový komplex metabolismus   MeSH
• respirační komplex III metabolismus   MeSH
• respirační komplex IV metabolismus   MeSH
• stárnutí metabolismus   MeSH
• svalové mitochondrie enzymologie   MeSH
• tělesná teplota fyziologie   MeSH
• transport elektronů fyziologie   MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH