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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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-Villar^a ; ^b ; ^c ; ¹ ; A. Rivera-Barahona^a ; ^b ; ^c ; ¹ ; C. Cuevas-Mart&iacute ; n^a ; ^b ; A. Guenzel^d ; B. Pé ; rez^a ; ^b ; ^c ; M.A. Barry^d ; M.P. Murphy^e ; A. Logan^e ; A. Gonzalez-Quintana^b ; ^f ; M.A. Mart&iacute ; n^b ; ^f ; S. Medina^g ; A. Gil-Izquierdo^g ; J.M. Cuezva^a ; ^b ; E. Richard^a ; ^b ; ^c ; ² ; L.R. Desviat^a ; ^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

•: We analyze mitochondrial function and redox homeostasis in a propionic acidemia mouse model.
•: Alterations in OXPHOS complexes and/or activities, and mtDNA depletion were present.
•: Increase in superoxide anion production and H₂O₂ levels, variations in antioxidant defences and lipid oxidative damage were also observed.
•: Mitochondrial dysfunction and redox imbalance probably contribute to the pathophysiology of propionic acidemia.