000060728 001__ 60728
000060728 005__ 20200221144321.0
000060728 0247_ $$2doi$$a10.1289/EHP182
000060728 0248_ $$2sideral$$a98278
000060728 037__ $$aART-2016-98278
000060728 041__ $$aeng
000060728 100__ $$0(orcid)0000-0002-3217-1424$$aLópez-Gallardo, E.$$uUniversidad de Zaragoza
000060728 245__ $$aEffects of tributyltin chloride on cybrids with or without an ATP synthase pathologic mutation
000060728 260__ $$c2016
000060728 5060_ $$aAccess copy available to the general public$$fUnrestricted
000060728 5203_ $$aBackground: The oxidative phosphorylation system (OXPHOS) includes nuclear chromosome (nDNA)– and mitochondrial DNA (mtDNA)–encoded polypeptides. Many rare OXPHOS disorders, such as striatal necrosis syndromes, are caused by genetic mutations. Despite important advances in sequencing procedures, causative mutations remain undetected in some patients. It is possible that etiologic factors, such as environmental toxins, are the cause of these cases. Indeed, the inhibition of a particular enzyme by a poison could imitate the biochemical effects of pathological mutations in that enzyme. Moreover, environmental factors can modify the penetrance or expressivity of pathological mutations. Objectives: We studied the interaction between mitochondrially encoded ATP synthase 6 (p.MT-ATP6) subunit and an environmental exposure that may contribute phenotypic differences between healthy individuals and patients suffering from striatal necrosis syndromes or other mitochondriopathies. Methods: We analyzed the effects of the ATP synthase inhibitor tributyltin chloride (TBTC), a widely distributed environmental factor that contaminates human food and water, on transmitochondrial cell lines with or without an ATP synthase mutation that causes striatal necrosis syndrome. Doses were selected based on TBTC concentrations previously reported in human whole blood samples. Results: TBTC modified the phenotypic effects caused by a pathological mtDNA mutation. Interestingly, wild-type cells treated with this xenobiotic showed similar bioenergetics when compared with the untreated mutated cells. Conclusions: In addition to the known genetic causes, our findings suggest that environmental exposure to TBTC might contribute to the etiology of striatal necrosis syndromes.
000060728 536__ $$9info:eu-repo/grantAgreement/ES/DGA/B33$$9info:eu-repo/grantAgreement/ES/FIS/FI12/00217$$9info:eu-repo/grantAgreement/ES/FIS/PI14-00070
000060728 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000060728 590__ $$a9.776$$b2016
000060728 591__ $$aENVIRONMENTAL SCIENCES$$b4 / 229 = 0.017$$c2016$$dQ1$$eT1
000060728 591__ $$aTOXICOLOGY$$b2 / 92 = 0.022$$c2016$$dQ1$$eT1
000060728 591__ $$aPUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH$$b4 / 176 = 0.023$$c2016$$dQ1$$eT1
000060728 592__ $$a3.13$$b2016
000060728 593__ $$aPublic Health, Environmental and Occupational Health$$c2016$$dQ1
000060728 593__ $$aHealth, Toxicology and Mutagenesis$$c2016$$dQ1
000060728 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000060728 700__ $$0(orcid)0000-0002-4379-1100$$aLlobet, L.$$uUniversidad de Zaragoza
000060728 700__ $$0(orcid)0000-0001-5964-6138$$aEmperador, S.
000060728 700__ $$0(orcid)0000-0003-1770-6299$$aMontoya, J.$$uUniversidad de Zaragoza
000060728 700__ $$0(orcid)0000-0002-0269-7337$$aRuiz-Pesini, E.$$uUniversidad de Zaragoza
000060728 7102_ $$11003$$2443$$aUniversidad de Zaragoza$$bDpto. Anatom.Histolog.Humanas$$cArea Histología
000060728 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole.
000060728 773__ $$g124, 9 (2016), 1399-1405$$pEnviron. health perspect.$$tENVIRONMENTAL HEALTH PERSPECTIVES$$x0091-6765
000060728 8564_ $$s2107331$$uhttp://zaguan.unizar.es/record/60728/files/texto_completo.pdf$$yVersión publicada
000060728 8564_ $$s144776$$uhttp://zaguan.unizar.es/record/60728/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000060728 909CO $$ooai:zaguan.unizar.es:60728$$particulos$$pdriver
000060728 951__ $$a2020-02-21-13:41:28
000060728 980__ $$aARTICLE