000077168 001__ 77168 000077168 005__ 20200716101513.0 000077168 0247_ $$2doi$$a10.1038/s41598-018-37542-2 000077168 0248_ $$2sideral$$a110375 000077168 037__ $$aART-2019-110375 000077168 041__ $$aeng 000077168 100__ $$aMontero, R. 000077168 245__ $$aPlasma coenzyme Q10 status is impaired in selected genetic conditions 000077168 260__ $$c2019 000077168 5060_ $$aAccess copy available to the general public$$fUnrestricted 000077168 5203_ $$aIdentifying diseases displaying chronic low plasma Coenzyme Q10 (CoQ) values may be important to prevent possible cardiovascular dysfunction. The aim of this study was to retrospectively evaluate plasma CoQ concentrations in a large cohort of pediatric and young adult patients. We evaluated plasma CoQ values in 597 individuals (age range 1 month to 43 years, average 11 years), studied during the period 2005-2016. Patients were classified into 6 different groups: control group of healthy participants, phenylketonuric patients (PKU), patients with mucopolysaccharidoses (MPS), patients with other inborn errors of metabolism (IEM), patients with neurogenetic diseases, and individuals with neurological diseases with no genetic diagnosis. Plasma total CoQ was measured by reverse-phase high-performance liquid chromatography with electrochemical detection and ultraviolet detection at 275¿nm. ANOVA with Bonferroni correction showed that plasma CoQ values were significantly lower in the PKU and MPS groups than in controls and neurological patients. The IEM group showed intermediate values that were not significantly different from those of the controls. In PKU patients, the Chi-Square test showed a significant association between having low plasma CoQ values and being classic PKU patients. The percentage of neurogenetic and other neurological patients with low CoQ values was low (below 8%). In conclusión, plasma CoQ monitoring in selected groups of patients with different IEM (especially in PKU and MPS patients, but also in IEM under protein-restricted diets) seems advisable to prevent the possibility of a chronic blood CoQ suboptimal status in such groups of patients. 000077168 536__ $$9info:eu-repo/grantAgreement/ES/ISCIII-FIS/PI17-00109$$9info:eu-repo/grantAgreement/ES/ISCIII-FIS/PI15-01082$$9info:eu-repo/grantAgreement/ES/ISCIII-FIS/PI15-00166$$9info:eu-repo/grantAgreement/ES/ISCIII/PI17-00021$$9info:eu-repo/grantAgreement/ES/DGA/B33-17R 000077168 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/ 000077168 590__ $$a3.998$$b2019 000077168 591__ $$aMULTIDISCIPLINARY SCIENCES$$b17 / 71 = 0.239$$c2019$$dQ1$$eT1 000077168 592__ $$a1.341$$b2019 000077168 593__ $$aMultidisciplinary$$c2019$$dQ1 000077168 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion 000077168 700__ $$aYubero, D. 000077168 700__ $$aSalgado, M.C. 000077168 700__ $$aGonzález, M.J. 000077168 700__ $$aCampistol, J. 000077168 700__ $$aO''Callaghan, M.D.M. 000077168 700__ $$aPineda, M. 000077168 700__ $$aDelgadillo, V. 000077168 700__ $$aMaynou, J. 000077168 700__ $$aFernandez, G. 000077168 700__ $$0(orcid)0000-0003-1770-6299$$aMontoya, J.$$uUniversidad de Zaragoza 000077168 700__ $$0(orcid)0000-0002-0269-7337$$aRuiz-Pesini, E.$$uUniversidad de Zaragoza 000077168 700__ $$aMeavilla, S. 000077168 700__ $$aNeergheen, V. 000077168 700__ $$aGarcía-Cazorla, A. 000077168 700__ $$aNavas, P. 000077168 700__ $$aHargreaves, I. 000077168 700__ $$aArtuch, R. 000077168 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole. 000077168 773__ $$g9, 1 (2019), 793 [8 pp]$$pSci. rep.$$tScientific Reports$$x2045-2322 000077168 8564_ $$s261312$$uhttps://zaguan.unizar.es/record/77168/files/texto_completo.pdf$$yVersión publicada 000077168 8564_ $$s113505$$uhttps://zaguan.unizar.es/record/77168/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada 000077168 909CO $$ooai:zaguan.unizar.es:77168$$particulos$$pdriver 000077168 951__ $$a2020-07-16-09:20:12 000077168 980__ $$aARTICLE