Abnormalities of hydrogen sulfide and glutathione pathways in mitochondrial dysfunction
Metadatos
Mostrar el registro completo del ítemEditorial
Elsevier
Materia
Coenzyme Q Mitochondria Reactive oxygen species (ROS) Oxidative stress Glutathione
Fecha
2021-01Referencia bibliográfica
C.M Quinzii, L.C Lopez. Abnormalities of hydrogen sulfide and glutathione pathways in mitochondrial dysfunction. Journal of Advanced Research 27 (2021) 79–84 [https://doi.org/10.1016/j.jare.2020.04.002]
Patrocinador
United States Department of Health & Human Services National Institutes of Health (NIH) - USA P01 HD080642; Ministerio de Ciencia e inn (LCL)Resumen
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.