130321 20240124152850.0 doi 10.1016/j.redox.2021.101871 sideral 122369 ART-2021-122369 eng Bayona-Bafaluy, M.P. Universidad de Zaragoza (orcid)0000-0002-8585-6371 Down syndrome is an oxidative phosphorylation disorder 2021 Down syndrome is the most common genomic disorder of intellectual disability and is caused by trisomy of chromosome 21. Several genes in this chromosome repress mitochondrial biogenesis. The goal of this study was to evaluate whether early overexpression of these genes may cause a prenatal impairment of oxidative phosphorylation negatively affecting neurogenesis. Reduction in the mitochondrial energy production and a lower mitochondrial function have been reported in diverse tissues or cell types, and also at any age, including early fetuses, suggesting that a defect in oxidative phosphorylation is an early and general event in Down syndrome individuals. Moreover, many of the medical conditions associated with Down syndrome are also frequently found in patients with oxidative phosphorylation disease. Several drugs that enhance mitochondrial biogenesis are nowadays available and some of them have been already tested in mouse models of Down syndrome restoring neurogenesis and cognitive defects. Because neurogenesis relies on a correct mitochondrial function and critical periods of brain development occur mainly in the prenatal and early neonatal stages, therapeutic approaches intended to improve oxidative phosphorylation should be provided in these periods. Access copy available to the general public Unrestricted info:eu-repo/grantAgreement/ES/DGA/B33-17R info:eu-repo/grantAgreement/ES/FIS/PI17-00021 info:eu-repo/grantAgreement/ES/FIS/PI17-00166 info:eu-repo/semantics/openAccess by-nc-nd http://creativecommons.org/licenses/by-nc-nd/3.0/es/ 10.787 2021 BIOCHEMISTRY & MOLECULAR BIOLOGY 27 / 297 = 0.091 2021 Q1 T1 2.191 2021 Clinical Biochemistry 2021 Q1 Biochemistry 2021 Q1 16.4 2021 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Garrido-Pérez, N. Universidad de Zaragoza (orcid)0000-0003-0145-3020 Meade, P. Universidad de Zaragoza (orcid)0000-0002-3587-6622 Iglesias, E. (orcid)0000-0003-1508-6516 Jiménez-Salvador, I. Universidad de Zaragoza (orcid)0000-0002-1426-3958 Montoya, J. Universidad de Zaragoza (orcid)0000-0003-1770-6299 Martínez-Cué, C. Ruiz-Pesini, E. Universidad de Zaragoza (orcid)0000-0002-0269-7337 1002 060 Universidad de Zaragoza Dpto. Bioq.Biolog.Mol. Celular Área Bioquímica y Biolog.Mole. 41 (2021), 101871 [12 pp] Redox biol. Redox Biology 2213-2317 6284910 http://zaguan.unizar.es/record/130321/files/texto_completo.pdf Versión publicada 2460418 http://zaguan.unizar.es/record/130321/files/texto_completo.jpg?subformat=icon icon Versión publicada oai:zaguan.unizar.es:130321 articulos driver 2024-01-24-15:02:57 ARTICLE