Comparison between the formation and the oxidation of dicarboxylylcarnitine esters in rat liver and skeletal muscle: possible implications for human inborn disorders of mitochondrial beta-oxidation.
Comparison between the formation and the oxidation of dicarboxylylcarnitine esters in rat liver and skeletal muscle: possible implications for human inborn disorders of mitochondrial beta-oxidation.
复制标题
大鼠肝脏和骨骼肌中二羧基肉碱酯的形成和氧化的比较：对人类先天性线粒体β-氧化疾病的可能影响。
DOI：
10.1007/bf01805531
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发表时间：
1989
影响因子：
4.2
通讯作者：
Draye,JP
中科院分区：
文献类型：
--
作者：
Vamecq,J;Draye,JP
在添加洗涤剂的情况下，在大鼠骨骼肌中检测到先前报道的大鼠肝脏中二羧基辅酶a合成酶的活性。在两种组织中都记录了肉碱二羧基转移酶的活性，但它们的底物链长度特异性不同。在大鼠骨骼肌中，而不是肝脏中，通过分光光度法测定底物依赖性铁氰化物还原，很容易检测到完整线粒体对十二烷二酰辅酶a的肉毒碱依赖性氧化。本文讨论了当前数据对尿二羧基左旋肉碱酯过量的发病机制的影响。
The activity of dicarboxylyl-CoA synthetase previously reported in rat liver was detected, in the presence of added detergents, in rat skeletal muscle. In both tissues, carnitine dicarboxylyltransferase activities were recorded but their substrate chain length specificity was, however, different. In rat skeletal muscle, but not liver, a carnitine-dependent oxidation by intact mitochondria of dodecanedioyl-CoA was easily detected by the spectrophotometric measurement of the substrate-dependent ferricyanide reduction.The implications of the present data for the pathogenesis of disorders with excess urine dicarboxylylcarnitine esters are discussed.