249 related articles for article (PubMed ID: 20153825)
1. Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts.
Morán M; Rivera H; Sánchez-Aragó M; Blázquez A; Merinero B; Ugalde C; Arenas J; Cuezva JM; Martín MA
Biochim Biophys Acta; 2010 May; 1802(5):443-53. PubMed ID: 20153825
[TBL] [Abstract][Full Text] [Related]
2. Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency.
Leipnitz G; Mohsen AW; Karunanidhi A; Seminotti B; Roginskaya VY; Markantone DM; Grings M; Mihalik SJ; Wipf P; Van Houten B; Vockley J
Sci Rep; 2018 Jan; 8(1):1165. PubMed ID: 29348607
[TBL] [Abstract][Full Text] [Related]
3. Rescue from galactose-induced death of Leigh Syndrome patient cells by pyruvate and NAD
Iannetti EF; Smeitink JAM; Willems PHGM; Beyrath J; Koopman WJH
Cell Death Dis; 2018 Nov; 9(11):1135. PubMed ID: 30429455
[TBL] [Abstract][Full Text] [Related]
4. Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy.
Ke BX; Pepe S; Grubb DR; Komen JC; Laskowski A; Rodda FA; Hardman BM; Pitt JJ; Ryan MT; Lazarou M; Koleff J; Cheung MM; Smolich JJ; Thorburn DR
Proc Natl Acad Sci U S A; 2012 Apr; 109(16):6165-70. PubMed ID: 22474353
[TBL] [Abstract][Full Text] [Related]
5. Broad phenotypic variability in patients with complex I deficiency due to mutations in NDUFS1 and NDUFV1.
Björkman K; Sofou K; Darin N; Holme E; Kollberg G; Asin-Cayuela J; Holmberg Dahle KM; Oldfors A; Moslemi AR; Tulinius M
Mitochondrion; 2015 Mar; 21():33-40. PubMed ID: 25615419
[TBL] [Abstract][Full Text] [Related]
6. Isolated mitochondrial complex I deficiency: explorative data analysis of patient cell parameters.
Blanchet L; Buydens MC; Smeitink JA; Willems PH; Koopman WJ
Curr Pharm Des; 2011 Dec; 17(36):4023-33. PubMed ID: 22188452
[TBL] [Abstract][Full Text] [Related]
7. Progressive cavitating leukoencephalopathy associated with respiratory chain complex I deficiency and a novel mutation in NDUFS1.
Ferreira M; Torraco A; Rizza T; Fattori F; Meschini MC; Castana C; Go NE; Nargang FE; Duarte M; Piemonte F; Dionisi-Vici C; Videira A; Vilarinho L; Santorelli FM; Carrozzo R; Bertini E
Neurogenetics; 2011 Feb; 12(1):9-17. PubMed ID: 21203893
[TBL] [Abstract][Full Text] [Related]
8. Changes in Mitochondrial Morphology and Bioenergetics in Human Lymphoblastoid Cells With Four Novel OPA1 Mutations.
Kao SH; Yen MY; Wang AG; Yeh YL; Lin AL
Invest Ophthalmol Vis Sci; 2015 Apr; 56(4):2269-78. PubMed ID: 25744979
[TBL] [Abstract][Full Text] [Related]
9. Modulation of oxidative phosphorylation and redox homeostasis in mitochondrial NDUFS4 deficiency via mesenchymal stem cells.
Melcher M; Danhauser K; Seibt A; Degistirici Ö; Baertling F; Kondadi AK; Reichert AS; Koopman WJH; Willems PHGM; Rodenburg RJ; Mayatepek E; Meisel R; Distelmaier F
Stem Cell Res Ther; 2017 Jun; 8(1):150. PubMed ID: 28646906
[TBL] [Abstract][Full Text] [Related]
10. Warburg-like effect is a hallmark of complex I assembly defects.
Desquiret-Dumas V; Leman G; Wetterwald C; Chupin S; Lebert A; Khiati S; Le Mao M; Geffroy G; Kane MS; Chevrollier A; Goudenege D; Gadras C; Tessier L; Barth M; Leruez S; Amati-Bonneau P; Henrion D; Bonneau D; Procaccio V; Reynier P; Lenaers G; Gueguen N
Biochim Biophys Acta Mol Basis Dis; 2019 Sep; 1865(9):2475-2489. PubMed ID: 31121247
[TBL] [Abstract][Full Text] [Related]
11. Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?
Koopman WJ; Verkaart S; Visch HJ; van Emst-de Vries S; Nijtmans LG; Smeitink JA; Willems PH
Am J Physiol Cell Physiol; 2007 Jul; 293(1):C22-9. PubMed ID: 17428841
[TBL] [Abstract][Full Text] [Related]
12. Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.
Breuer ME; Willems PH; Smeitink JA; Koopman WJ; Nooteboom M
IUBMB Life; 2013 Mar; 65(3):202-8. PubMed ID: 23378164
[TBL] [Abstract][Full Text] [Related]
13. Assembly defects induce oxidative stress in inherited mitochondrial complex I deficiency.
Leman G; Gueguen N; Desquiret-Dumas V; Kane MS; Wettervald C; Chupin S; Chevrollier A; Lebre AS; Bonnefont JP; Barth M; Amati-Bonneau P; Verny C; Henrion D; Bonneau D; Reynier P; Procaccio V
Int J Biochem Cell Biol; 2015 Aug; 65():91-103. PubMed ID: 26024641
[TBL] [Abstract][Full Text] [Related]
14. Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency.
Ugalde C; Janssen RJ; van den Heuvel LP; Smeitink JA; Nijtmans LG
Hum Mol Genet; 2004 Mar; 13(6):659-67. PubMed ID: 14749350
[TBL] [Abstract][Full Text] [Related]
15. Exercise intolerance and developmental delay associated with a novel mitochondrial ND5 mutation.
Fang H; Shi H; Li X; Sun D; Li F; Li B; Ding Y; Ma Y; Liu Y; Zhang Y; Shen L; Bai Y; Yang Y; Lu J
Sci Rep; 2015 May; 5():10480. PubMed ID: 26014388
[TBL] [Abstract][Full Text] [Related]
16. Leigh syndrome associated with mitochondrial complex I deficiency due to novel mutations In NDUFV1 and NDUFS2.
Marin SE; Mesterman R; Robinson B; Rodenburg RJ; Smeitink J; Tarnopolsky MA
Gene; 2013 Mar; 516(1):162-7. PubMed ID: 23266820
[TBL] [Abstract][Full Text] [Related]
17. Defective mitochondrial fusion, altered respiratory function, and distorted cristae structure in skin fibroblasts with heterozygous OPA1 mutations.
Agier V; Oliviero P; Lainé J; L'Hermitte-Stead C; Girard S; Fillaut S; Jardel C; Bouillaud F; Bulteau AL; Lombès A
Biochim Biophys Acta; 2012 Oct; 1822(10):1570-80. PubMed ID: 22800932
[TBL] [Abstract][Full Text] [Related]
18. Deficiency in mitochondrial complex I activity due to Ndufs6 gene trap insertion induces renal disease.
Forbes JM; Ke BX; Nguyen TV; Henstridge DC; Penfold SA; Laskowski A; Sourris KC; Groschner LN; Cooper ME; Thorburn DR; Coughlan MT
Antioxid Redox Signal; 2013 Aug; 19(4):331-43. PubMed ID: 23320803
[TBL] [Abstract][Full Text] [Related]
19. Mitochondrial energetics is impaired in very long-chain acyl-CoA dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers.
Seminotti B; Leipnitz G; Karunanidhi A; Kochersperger C; Roginskaya VY; Basu S; Wang Y; Wipf P; Van Houten B; Mohsen AW; Vockley J
Hum Mol Genet; 2019 Mar; 28(6):928-941. PubMed ID: 30445591
[TBL] [Abstract][Full Text] [Related]
20. The Putative Drp1 Inhibitor mdivi-1 Is a Reversible Mitochondrial Complex I Inhibitor that Modulates Reactive Oxygen Species.
Bordt EA; Clerc P; Roelofs BA; Saladino AJ; Tretter L; Adam-Vizi V; Cherok E; Khalil A; Yadava N; Ge SX; Francis TC; Kennedy NW; Picton LK; Kumar T; Uppuluri S; Miller AM; Itoh K; Karbowski M; Sesaki H; Hill RB; Polster BM
Dev Cell; 2017 Mar; 40(6):583-594.e6. PubMed ID: 28350990
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]