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首页> 外文期刊>Biochimica et biophysica acta. Molecular basis of disease: BBA >Myocardial carnitine and carnitine palmitoyltransferase deficiencies in patients with severs heart failure
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Myocardial carnitine and carnitine palmitoyltransferase deficiencies in patients with severs heart failure

机译：患有心力衰竭的患者的心肌肉碱和肉碱棕榈酰转移酶缺乏症

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摘要

We studied myocardial tissue from 25 cardiac transplant recipients, who had end-stage congestive heart failure (CHF), and from 21 control donor hearts. Concentrations of total carnitine (TC), free carnitine (FC), short-chain acylcarnitines, long-chain acylcarnitines (LACA) as well as carnitine palmitoyltransferase (CPT) activities were measured in myocardial tissue homogenates and referred to the concentration of non-collagen protein. Compared to controls, the concentrations of TC and FC as well as total CPT activities were significantly lower in patients. LCAC levels and the LCAC to FC ratio values were significantly greater in patients than in controls. While the malonyl-CoA sensitive fraction of CPT, which represents CPT I activity, was similar in patients and controls, the residual CPT activity after inhibition by malonyl-CoA, representing CPT II activity, was significantly reduced in patients compared to controls. Moreover, the activity of CPT in the presence of Triton X-100, which also represents the activity of CPT II, was significantly lower in patients than in controls. Malonyl-CoA concentrations required for half-maximal inhibition of CPT activity were significantly greater in patients than in controls. There was a linear relationship between ejection fraction (EF) values and concentrations of TC, FC, or total CPT activities. Values for LCAC and the LCAC to FC ratio were inversely related to EF values. We conclude that failing heart shows decreased total CPT and CPT II activities and carnitine deficiency than may be related to ventricle function.
机译：我们研究了来自25名患有晚期充血性心力衰竭（CHF）的心脏移植受者和21个对照供体心脏的心肌组织。测量心肌组织匀浆中总肉碱（TC），游离肉碱（FC），短链酰基肉碱，长链酰基肉碱（LACA）以及肉碱棕榈酰转移酶（CPT）的浓度，并指非胶原蛋白的浓度蛋白。与对照组相比，患者的TC和FC浓度以及总CPT活性均显着降低。患者的LCAC水平和LCAC与FC的比值显着高于对照组。尽管代表患者CPT I活性的CPT丙二酰辅酶A敏感性部分在患者和对照组中相似，但是与对照相比，代表丙二肽II活性的丙二酰辅酶A抑制后的残余CPT活性显着降低。此外，在Triton X-100存在下，CPT的活性（也代表CPT II的活性）在患者中明显低于对照组。患者半数抑制CPT活性所需的丙二酰辅酶A浓度显着高于对照组。射血分数（EF）值与TC，FC或总CPT活性的浓度之间存在线性关系。 LCAC和LCAC与FC的值与EF值成反比。我们得出的结论是，与可能与心室功能有关的心脏衰竭相比，总的CPT和CPT II活性降低以及肉碱缺乏程度降低。

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