@misc{10481/58176,
year = {2017},
url = {http://hdl.handle.net/10481/58176},
abstract = {Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several other functions in the cellular metabolism. One of them is to function as an electron carrier in the reaction catalyzed by sulfide:quinone oxidoreductase (SQR), which catalyzes the first reaction in the hydrogen sulfide oxidation pathway. Therefore, SQR may be affected by CoQ deficiency. Using human skin fibroblasts and two mouse models with primary CoQ deficiency, we demonstrate that severe CoQ deficiency causes a reduction in SQR levels and activity, which leads to an alteration of mitochondrial sulfide metabolism. In cerebrum of Coq9R239X mice, the deficit in SQR induces an increase in thiosulfate sulfurtransferase and sulfite oxidase, as well as modifications in the levels of thiols. As a result, biosynthetic pathways of glutamate, serotonin, and catecholamines were altered in the cerebrum, and the blood pressure was reduced. Therefore, this study reveals the reduction in SQR activity as one of the pathomechanisms associated with CoQ deficiency syndrome.},
organization = {This work was supported by grants from Ministerio de Economía y Competitividad, Spain, and the ERDF (SAF2013-47761-R, SAF2014-55523-R, RD12/0042/0011 and SAF201565786-R), from the Consejería de Economía, Innovación, Ciencia y Empleo, Junta de Andalucía (P10-CTS-6133), from the NIH (P01HD080642) and from the foundation “todos somos raros, todos somos únicos”. MLS is a predoctoral fellow from the Consejería de Economía, Innovación, Ciencia y Empleo, Junta de Andalucía. LCL is supported by the “Ramón y Cajal” National Programme, Ministerio de Economía y Competitividad, Spain (RYC-2011-07643).},
publisher = {EMBO Press},
keywords = {Blood pressure},
keywords = {Glutathione},
keywords = {Mitochondrial disease},
keywords = {SQR},
title = {CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome},
author = {Luna-Sánchez, Marta and Hidalgo Gutiérrez, Agustín and Chaves Serrano, Julio and Barriocanal Casado, Eliana and Santos Fandila, Ángela and Romero Pérez, Miguel and Sayed, Ramy K. A. and Duarte, Juan and Escames Rosa, Germaine and Acuña Castroviejo, Darío and Cuadros López, José Luis},
}