Wenchich László, Zeman Jirí, Hansíková Hana, Plavka Richard, Sperl Wolfgang, Houstek Josef
Department of Pediatrics, Center for Integrated Genomics, Charles University, Prague, Czech Republic.
Biol Neonate. 2002;81(4):229-35. doi: 10.1159/000056753.
The activity, amount and protein composition of pyruvate dehydrogenase (PDH) and respiratory chain complexes were studied in muscle mitochondria obtained postmortally from 6 neonates with a gestational age of 23-29 weeks. The activities of PDH and respiratory chain complex III and IV and citrate synthase were significantly lower in comparison with control children aged 0.5-2 and 2-20 years. Protein analyses revealed a parallel decrease in the content of PDH, respiratory chain complexes and their subunits in the cases analyzed. The observed immaturity of the mitochondrial energy-providing system suggests that significant development of mitochondrial energy metabolism occurs during the last 3 months of prenatal development. The metabolic disturbances of mitochondrial energy conversion associated with the low functional capacity and content of PDH and respiratory chain complexes may play an important role in the morbidity of very premature neonates.
对6例胎龄为23 - 29周的新生儿死后获取的肌肉线粒体中丙酮酸脱氢酶(PDH)以及呼吸链复合物的活性、含量和蛋白质组成进行了研究。与0.5 - 2岁以及2 - 20岁的对照儿童相比,PDH、呼吸链复合物III和IV以及柠檬酸合酶的活性显著降低。蛋白质分析显示,在所分析的病例中,PDH、呼吸链复合物及其亚基的含量呈平行下降。观察到的线粒体能量供应系统的不成熟表明,线粒体能量代谢在产前发育的最后3个月有显著发展。与PDH和呼吸链复合物的低功能能力及含量相关的线粒体能量转换代谢紊乱,可能在极早产儿的发病过程中起重要作用。