In vivo evidence of mitochondrial dysfunction and altered redox homeostasis in a genetic mouse model of propionic acidemia: Implications for the pathophysiology of this disorder
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- 作者：L. Gallego-Villara ; b ; c ; 1 ; A. Rivera-Barahonaa ; b ; c ; 1 ; C. Cuevas-Martí ; na ; b ; A. Guenzeld ; B. Pé ; reza ; b ; c ; M.A. Barryd ; M.P. Murphye ; A. Logane ; A. Gonzalez-Quintanab ; f ; M.A. Martí ; nb ; f ; S. Medinag ; A. Gil-Izquierdog ; J.M. Cuezvaa ; b ; E. Richarda ; b ; c ; 2 ; L.R. Desviata ; b ; c ; 2 ; lruiz@cbm.csic.es" class="auth_mail" title="E-mail the corresponding author
- 关键词：βF1 ; ATP synthase ; F1 complex ; beta polypeptide ; BNP ; brain natriuretic peptide ; C-III ; complex III ; COX-II ; cytochrome c oxidase subunit II ; COX-IV ; cytochrome c oxidase subunit IV ; DAPI ; 4&prime ; 6-diamidino-2-phenylindole ; DHE ; dihydroethidium ; GPx1 ; glutathione peroxidase ; HADHA ; hydroxyacyl-CoA dehydrogenase ; alpha subunit ; IF1 ; ATPase inhibitory factor 1 ; IsoPs ; Isoprostanes ; LDHA ; lactate dehydrogenase A ; MDA ; malondialdehyde ; MMA ; methylmalonic academia ; NDUFS3 ; NADH dehydrogenase (ubiqu
- 刊名：Free Radical Biology and Medicine
- 出版年：2016
- 出版时间：July 2016
- 年：2016
- 卷：96
- 期：Complete
- 页码：1-12
- 全文大小：1984 K
文摘
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We analyze mitochondrial function and redox homeostasis in a propionic acidemia mouse model.
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Alterations in OXPHOS complexes and/or activities, and mtDNA depletion were present.
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Increase in superoxide anion production and H2O2 levels, variations in antioxidant defences and lipid oxidative damage were also observed.
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Mitochondrial dysfunction and redox imbalance probably contribute to the pathophysiology of propionic acidemia.