198 related articles for article (PubMed ID: 25620325)
1. Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
Blanchet L; Smeitink JA; van Emst-de Vries SE; Vogels C; Pellegrini M; Jonckheere AI; Rodenburg RJ; Buydens LM; Beyrath J; Willems PH; Koopman WJ
Sci Rep; 2015 Jan; 5():8035. PubMed ID: 25620325
[TBL] [Abstract][Full Text] [Related]
2. 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]
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. Cell-Permeable Succinate Increases Mitochondrial Membrane Potential and Glycolysis in Leigh Syndrome Patient Fibroblasts.
Bakare AB; Rao RR; Iyer S
Cells; 2021 Aug; 10(9):. PubMed ID: 34571904
[TBL] [Abstract][Full Text] [Related]
5. 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]
6. The effect of small molecules on nuclear-encoded translation diseases.
Soiferman D; Ayalon O; Weissman S; Saada A
Biochimie; 2014 May; 100():184-91. PubMed ID: 24012549
[TBL] [Abstract][Full Text] [Related]
7. The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome.
Kanabus M; Fassone E; Hughes SD; Bilooei SF; Rutherford T; Donnell MO; Heales SJR; Rahman S
J Inherit Metab Dis; 2016 May; 39(3):415-426. PubMed ID: 27080638
[TBL] [Abstract][Full Text] [Related]
8. Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice.
Frambach SJCM; van de Wal MAE; van den Broek PHH; Smeitink JAM; Russel FGM; de Haas R; Schirris TJJ
Biochim Biophys Acta Mol Basis Dis; 2020 Jun; 1866(6):165727. PubMed ID: 32070771
[TBL] [Abstract][Full Text] [Related]
9. A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome.
Assouline Z; Jambou M; Rio M; Bole-Feysot C; de Lonlay P; Barnerias C; Desguerre I; Bonnemains C; Guillermet C; Steffann J; Munnich A; Bonnefont JP; Rötig A; Lebre AS
Biochim Biophys Acta; 2012 Jun; 1822(6):1062-9. PubMed ID: 22326555
[TBL] [Abstract][Full Text] [Related]
10. 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]
11. 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]
12. Infantile mitochondrial disorders.
Carrozzo R; Piemonte F; Tessa A; Lucioli S; Rizza T; Meschini MC; Fattori F; Santorelli FM
Biosci Rep; 2007 Jun; 27(1-3):105-12. PubMed ID: 17486440
[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. The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency.
Distelmaier F; Visch HJ; Smeitink JA; Mayatepek E; Koopman WJ; Willems PH
J Mol Med (Berl); 2009 May; 87(5):515-22. PubMed ID: 19255735
[TBL] [Abstract][Full Text] [Related]
15. N-acetylcysteine and vitamin E rescue animal longevity and cellular oxidative stress in pre-clinical models of mitochondrial complex I disease.
Polyak E; Ostrovsky J; Peng M; Dingley SD; Tsukikawa M; Kwon YJ; McCormack SE; Bennett M; Xiao R; Seiler C; Zhang Z; Falk MJ
Mol Genet Metab; 2018 Apr; 123(4):449-462. PubMed ID: 29526616
[TBL] [Abstract][Full Text] [Related]
16. NDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy.
Piekutowska-Abramczuk D; Assouline Z; Mataković L; Feichtinger RG; Koňařiková E; Jurkiewicz E; Stawiński P; Gusic M; Koller A; Pollak A; Gasperowicz P; Trubicka J; Ciara E; Iwanicka-Pronicka K; Rokicki D; Hanein S; Wortmann SB; Sperl W; Rötig A; Prokisch H; Pronicka E; Płoski R; Barcia G; Mayr JA
Am J Hum Genet; 2018 Mar; 102(3):460-467. PubMed ID: 29429571
[TBL] [Abstract][Full Text] [Related]
17. 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]
18. Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain.
Kayser EB; Sedensky MM; Morgan PG
PLoS One; 2016; 11(1):e0148219. PubMed ID: 26824698
[TBL] [Abstract][Full Text] [Related]
19. 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]
20. Treatment of human cells derived from MERRF syndrome by peptide-mediated mitochondrial delivery.
Chang JC; Liu KH; Chuang CS; Su HL; Wei YH; Kuo SJ; Liu CS
Cytotherapy; 2013 Dec; 15(12):1580-96. PubMed ID: 24199594
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]