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Abnormalities of hydrogen sulfide and glutathione pathways in mitochondrial dysfunction
Journal of Advanced Research ( IF 10.7 ) Pub Date : 2020-04-07 , DOI: 10.1016/j.jare.2020.04.002
Catarina M Quinzii 1 , Luis C Lopez 2
Affiliation  

Background

Mitochondrial disorders are genetic diseases for which therapy remains woefully inadequate. Therapy of these disorders is particularly challenging partially due to the heterogeneity and tissue-specificity of pathomechanisms involved in these disorders. Abnormalities in hydrogen sulfide (H2S) metabolism are emerging as novel mechanism in mitochondrial dysfunction. However, further studies are necessary to understand the effects, protective or detrimental, of these abnormalities, and their relevance, in mitochondrial diseases.

Aim of Review

To review the recent evidences of derangement of the metabolism of H2S, at biosynthesis or oxidation levels, in mitochondrial dysfunction, focusing specifically on the alterations of H2S oxidation caused by primary Coenzyme Q (CoQ) deficiency.

Key Scientific Concepts of Review

Mitochondria play a key role in the regulation of H2S and GSH metabolism pathways. However, further studies are needed to understand the consequences of abnormalities of H2S and GSH synthesis on the oxidation pathway, and vice versa; and on the levels of H2S and GSH, their tissue-specific detrimental effects, and their role the role in mitochondrial diseases. Beside the known H2S pathways, additional, tissue-specific, enzymatic systems, involved in H2S production and elimination, might exist.



中文翻译:

线粒体功能障碍中的硫化氢和谷胱甘肽通路异常

背景

线粒体疾病是治疗仍然严重不足的遗传疾病。这些疾病的治疗特别具有挑战性,部分原因是这些疾病所涉及的病理机制的异质性和组织特异性。硫化氢 (H 2 S) 代谢异常正在成为线粒体功能障碍的新机制。然而,需要进一步的研究来了解这些异常的影响,保护性或有害性,以及它们在线粒体疾病中的相关性。

审查目的

回顾 H 2 S 代谢紊乱的最新证据,在生物合成或氧化水平上,在线粒体功能障碍中,特别关注由原发性辅酶 Q (CoQ) 缺乏引起的 H 2 S 氧化变化。

审查的关键科学概念

线粒体在调节 H 2 S 和 GSH 代谢途径中起关键作用。然而,需要进一步的研究来了解H 2 S和GSH合成异常对氧化途径的影响,反之亦然;以及 H 2 S 和 GSH 的水平、它们的组织特异性有害作用以及它们在线粒体疾病中的作用。除了已知的 H 2 S 途径之外,还可能存在参与 H 2 S 产生和消除的其他组织特异性酶促系统。

更新日期:2020-04-07
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