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Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention.
van de Wal MAE, Adjobo-Hermans MJW, Keijer J, Schirris TJJ, Homberg JR, Wieckowski MR, Grefte S, van Schothorst EM, van Karnebeek C, Quintana A, Koopman WJH. van de Wal MAE, et al. Among authors: wieckowski mr. Brain. 2022 Mar 29;145(1):45-63. doi: 10.1093/brain/awab426. Brain. 2022. PMID: 34849584 Free PMC article. Review.
Mitochondrial hyperpolarization during chronic complex I inhibition is sustained by low activity of complex II, III, IV and V.
Forkink M, Manjeri GR, Liemburg-Apers DC, Nibbeling E, Blanchard M, Wojtala A, Smeitink JA, Wieckowski MR, Willems PH, Koopman WJ. Forkink M, et al. Among authors: wieckowski mr. Biochim Biophys Acta. 2014 Aug;1837(8):1247-56. doi: 10.1016/j.bbabio.2014.04.008. Epub 2014 Apr 24. Biochim Biophys Acta. 2014. PMID: 24769419 Free article.
Mitochondrial dysfunction in primary human fibroblasts triggers an adaptive cell survival program that requires AMPK-α.
Distelmaier F, Valsecchi F, Liemburg-Apers DC, Lebiedzinska M, Rodenburg RJ, Heil S, Keijer J, Fransen J, Imamura H, Danhauser K, Seibt A, Viollet B, Gellerich FN, Smeitink JA, Wieckowski MR, Willems PH, Koopman WJ. Distelmaier F, et al. Among authors: wieckowski mr. Biochim Biophys Acta. 2015 Mar;1852(3):529-40. doi: 10.1016/j.bbadis.2014.12.012. Epub 2014 Dec 20. Biochim Biophys Acta. 2015. PMID: 25536029 Free article.
Quantifying ROS levels using CM-H2DCFDA and HyPer.
Oparka M, Walczak J, Malinska D, van Oppen LMPE, Szczepanowska J, Koopman WJH, Wieckowski MR. Oparka M, et al. Among authors: wieckowski mr. Methods. 2016 Oct 15;109:3-11. doi: 10.1016/j.ymeth.2016.06.008. Epub 2016 Jun 11. Methods. 2016. PMID: 27302663 Review.
NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4-/- mice and Leigh syndrome patients: A stabilizing role for NDUFAF2.
Adjobo-Hermans MJW, de Haas R, Willems PHGM, Wojtala A, van Emst-de Vries SE, Wagenaars JA, van den Brand M, Rodenburg RJ, Smeitink JAM, Nijtmans LG, Sazanov LA, Wieckowski MR, Koopman WJH. Adjobo-Hermans MJW, et al. Among authors: wieckowski mr. Biochim Biophys Acta Bioenerg. 2020 Aug 1;1861(8):148213. doi: 10.1016/j.bbabio.2020.148213. Epub 2020 Apr 23. Biochim Biophys Acta Bioenerg. 2020. PMID: 32335026 Free article.
Oxidative stress-dependent p66Shc phosphorylation in skin fibroblasts of children with mitochondrial disorders.
Lebiedzinska M, Karkucinska-Wieckowska A, Giorgi C, Karczmarewicz E, Pronicka E, Pinton P, Duszynski J, Pronicki M, Wieckowski MR. Lebiedzinska M, et al. Among authors: wieckowski mr. Biochim Biophys Acta. 2010 Jun-Jul;1797(6-7):952-60. doi: 10.1016/j.bbabio.2010.03.005. Epub 2010 Mar 10. Biochim Biophys Acta. 2010. PMID: 20226758 Free article.
Increased reactive oxygen species (ROS) production and low catalase level in fibroblasts of a girl with MEGDEL association (Leigh syndrome, deafness, 3-methylglutaconic aciduria).
Karkucinska-Wieckowska A, Lebiedzinska M, Jurkiewicz E, Pajdowska M, Trubicka J, Szymanska-Debinska T, Suski J, Pinton P, Duszynski J, Pronicki M, Wieckowski MR, Pronicka E. Karkucinska-Wieckowska A, et al. Among authors: wieckowski mr. Folia Neuropathol. 2011;49(1):56-63. Folia Neuropathol. 2011. PMID: 21455844 Free article.
Effect of mtDNA point mutations on cellular bioenergetics.
Szczepanowska J, Malinska D, Wieckowski MR, Duszynski J. Szczepanowska J, et al. Among authors: wieckowski mr. Biochim Biophys Acta. 2012 Oct;1817(10):1740-6. doi: 10.1016/j.bbabio.2012.02.028. Epub 2012 Mar 3. Biochim Biophys Acta. 2012. PMID: 22406627 Free article. Review.
165 results