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首页> 外文期刊>Canadian Journal of Physiology and Pharmacology >Na + –H + exchanger and proton channel in heart failure associated with Becker and Duchenne muscular dystrophies
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Na + –H + exchanger and proton channel in heart failure associated with Becker and Duchenne muscular dystrophies

机译：与Becker和Duchenne肌营养不良相关的心力衰竭Na + -H +交换器和质子通道

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摘要

Cardiomyopathy is found in patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies, which are linked muscle diseases caused by mutations in the dystrophin gene. Dystrophin defects are not limited to DMD but are also present in mild BMD. The hereditary cardiomyopathic hamster of the UM-X7.1 strain is a particular experimental model of heart failure (HF) leading to early death in muscular dystrophy (dystrophin deficiency and sarcoglycan mutation) and heart disease (δ-sarcoglycan deficiency and dystrophin mutation) in human DMD. Using this model, our previous work showed a defect in intracellular sodium homeostasis before the appearance of any apparent biochemical and histological defects. This was attributed to the continual presence of the fetal slow sodium channel, which was also found to be active in human DMD. Due to muscular intracellular acidosis, the intracellular sodium overload in DMD and BMD was also due to sodium influx through the sodium–hydrogen exchanger NHE-1. Lifetime treatment with an NHE-1 inhibitor prevented intracellular Na + overload and early death due to HF. Our previous work also showed that another proton transporter, the voltage-gated proton channel (Hv1), exists in many cell types including heart cells and skeletal muscle fibers. The Hv1 could be indirectly implicated in the beneficial effect of blocking NHE-1.
机译：心肌病见于Duchenne（DMD）和Becker（BMD）肌营养不良症患者，这是由肌营养不良蛋白基因突变引起的相关肌肉疾病。肌营养不良蛋白缺陷不仅限于DMD，也存在于轻度BMD中。UM-X7的遗传性心肌病仓鼠。1株是一种特殊的心力衰竭（HF）实验模型，导致人类DMD中肌营养不良（肌营养不良蛋白缺乏和肌聚糖突变）和心脏病（δ-肌聚糖缺乏和肌营养不良蛋白突变）的早期死亡。利用这个模型，我们之前的研究表明，在出现任何明显的生化和组织学缺陷之前，细胞内钠稳态存在缺陷。这归因于胎儿缓慢钠通道的持续存在，该通道在人类DMD中也被发现是活跃的。由于肌肉细胞内酸中毒，DMD和BMD中的细胞内钠超载也是由于钠通过钠-氢交换剂NHE-1流入。NHE-1抑制剂的终生治疗可防止细胞内钠超载和HF导致的早期死亡。我们之前的工作还表明，另一种质子转运蛋白，电压门控质子通道（Hv1），存在于许多细胞类型中，包括心脏细胞和骨骼肌纤维。Hv1可能间接参与阻断NHE-1的有益作用。

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