Smeitink JA

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Publications

 PublishedReference
Hoogstraten 2023 Arch Toxicol2023Hoogstraten CA, Jacobs MME, de Boer G, van de Wal MAE, Koopman WJH, Smeitink JAM, Russel FGM, Schirris TJJ (2023) Metabolic impact of genetic and chemical ADP/ATP carrier inhibition in renal proximal tubule epithelial cells. https://doi.org/10.1007/s00204-023-03510-7
Frambach 2020 Biochim Biophys Acta Mol Basis Dis2020Frambach SJCM, van de Wal MAE, van den Broek PHH, Smeitink JAM, Russel FGM, de Haas R, Schirris TJJ (2020) Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice. Biochim Biophys Acta Mol Basis Dis 1866:165727.
Friederich 2018 Nat Commun2018Friederich MW, Timal S, Powell CA, Dallabona C, Kurolap A, Palacios-Zambrano S, Bratkovic D, Derks TGJ, Bick D, Bouman K, Chatfield KC, Damouny-Naoum N, Dishop MK, Falik-Zaccai TC, Fares F, Fedida A, Ferrero I, Gallagher RC, Garesse R, Gilberti M, Gonzรกlez C, Gowan K, Habib C, Halligan RK, Kalfon L, Knight K, Lefeber D, Mamblona L, Mandel H, Mory A, Ottoson J, Paperna T, Pruijn GJM, Rebelo-Guiomar PF, Saada A, Sainz B Jr, Salvemini H, Schoots MH, Smeitink JA, Szukszto MJ, Ter Horst HJ, van den Brandt F, van Spronsen FJ, Veltman JA, Wartchow E, Wintjes LT, Zohar Y, Fernรกndez-Moreno MA, Baris HN, Donnini C, Minczuk M, Rodenburg RJ, Van Hove JLK (2018) Pathogenic variants in glutamyl-tRNAGln amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder. Nat Commun 9:4065.
Allard 2018 J Clin Endocrinol Metab2018Allard NAE, Schirris TJJ, Verheggen RJ, Russel FGM, Rodenburg RJ, Smeitink JAM, Thompson PD, Hopman MTE, Timmers S (2018) Statins affect skeletal muscle performance: evidence for disturbances in energy metabolism. J Clin Endocrinol Metab 103:75-84.
Koopman 2016 EMBO Mol Med2016Koopman WJ, Beyrath J, Fung CW, Koene S, Rodenburg RJ, Willems PH, Smeitink JA (2016) Mitochondrial disorders in children: toward development of small-molecule treatment strategies. EMBO Mol Med 8:311-27.
Iannetti 2016 Nat Protoc2016Iannetti EF, Smeitink JA, Beyrath J, Willems PH, Koopman WJ (2016) Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy. Nat Protoc 11:1693-710.
Schirris 2015 Cell Metab2015Schirris TJ, Renkema GH, Ritschel T, Voermans NC, Bilos A, van Engelen BG, Brandt U, Koopman WJ, Beyrath JD, Rodenburg RJ, Willems PH, Smeitink JA, Russel FG (2015) Statin-induced myopathy is associated with mitochondrial complex III inhibition. Cell Metab 22:399-407.
Blanchet 2015 Sci Rep2015Blanchet L, Smeitink JA, van Emst-de Vries SE, Vogels C, Pellegrini M, Jonckheere AI, Rodenburg RJ, Buydens LM, Beyrath J, Willems PH, Koopman WJ (2015) Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning. Sci Rep 5:8035.
Forkink 2014 Biochim Biophys Acta2014Forkink M, Manjeri GR, Liemburg-Apers DC, Nibbeling E, Blanchard M, Wojtala A, Smeitink JA, Wieckowski MR, Willems PH, Koopman WJ (2014) Mitochondrial hyperpolarization during chronic Complex I inhibition is sustained by low activity of Complex II, III, IV and V. Biochim Biophys Acta 1837:1247-56.
Breuer 2013 Neurobiol Dis2013Breuer ME, Koopman WJ, Koene S, Nooteboom M, Rodenburg RJ, Willems PH, Smeitink JA (2013) The role of mitochondrial OXPHOS dysfunction in the development of neurologic diseases. Neurobiol Dis 51:27-34. https://doi.org/10.1016/j.nbd.2012.03.007
Distelmaier 2012 Antioxid Redox Signal2012Distelmaier F, Valsecchi F, Forkink M, van Emst-de Vries S, Swarts HG, Rodenburg RJ, Verwiel ET, Smeitink JA, Willems PH, Koopman WJ (2012) Trolox-sensitive reactive oxygen species regulate mitochondrial morphology, oxidative phosphorylation and cytosolic calcium handling in healthy cells. Antioxid Redox Signal 17:1657-69.
Koopman 2012 N Engl J Med2012Koopman WJ, Willems PH, Smeitink JA (2012) Monogenic mitochondrial disorders. N Engl J Med 366:1132-41.
Koene 2011 J Inherit Metab Dis2011Koene S, Willems PH, Roestenberg P, Koopman WJ, Smeitink JA (2011) Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency. J Inherit Metab Dis 34:293-307. https://doi.org/10.1007/s10545-009-9005-x
Dieteren 2011 Proc Natl Acad Sci U S A2011Dieteren CE, Gielen SC, Nijtmans LG, Smeitink JA, Swarts HG, Brock R, Willems PH, Koopman WJ (2011) Solute diffusion is hindered in the mitochondrial matrix. Proc Natl Acad Sci U S A 108:8657-62.
Koopman 2010 Antioxid Redox Signal2010Koopman WJ, Nijtmans LG, Dieteren CE, Roestenberg P, Valsecchi F, Smeitink JA, Willems PH (2010) Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation. Antioxid Redox Signal 12:1431-70.
Distelmaier 2009 Brain2009Distelmaier F, Koopman WJ, van den Heuvel LP, Rodenburg RJ, Mayatepek E, Willems PH, Smeitink JA (2009) Mitochondrial Complex I deficiency: from organelle dysfunction to clinical disease. Brain 132:833-42.
Mortiboys 2008 Ann Neurol2008Mortiboys H, Thomas KJ, Koopman WJ, Klaffke S, Abou-Sleiman P, Olpin S, Wood NW, Willems PH, Smeitink JA, Cookson MR, Bandmann O (2008) Mitochondrial function and morphology are impaired in parkin-mutant fibroblasts. Ann Neurol 64:555-65.
Rorbach 2008 Nucleic Acids Res2008Rorbach J, Richter R, Wessels HJ, Wydro M, Pekalski M, Farhoud M, Kรผhl I, Gaisne M, Bonnefoy N, Smeitink JA, Lightowlers RN, Chrzanowska-Lightowlers ZMA (2008) The human mitochondrial ribosome recycling factor is essential for cell viability. Nucleic Acids Res 36:5787-99.

Abstracts

 PublishedReference
Koopman 2022 Abstract Bioblast20227.7. ยซ10+5ยป
Koopman Werner
Bulthuis EP, Einer C, Distelmaier F, Groh L, van Emst-de Vries SE, van de Westerlo E, van de Wal M, Wagenaars J, Rodenburg RJ, Smeitink JAM, Riksen NP, Willems PGHM, Adjobo-Hermans MJW, Zischka H, Koopman Werner JH (2022) The decylTPP mitochondria-targeting moiety lowers electron transport system supercomplex levels in primary human skin fibroblasts. Bioblast 2022: BEC Inaugural Conference. In: https://doi.org/10.26124/bec:2022-0001 ยปWatch the presentationยซ
Foriel 2015 Abstract MiP20152015Drosophila as a model to study therapeutic approaches for mitochondrial diseases.
Wojtala 2014 Abstract MiP20142014Proteomic analysis of mitochondrial Complex I deficient mouse model - impact of Complex I deficiency on p66Shc-Ser36 phosphorylation pathway in NDUFS4-/- mouse tissues.
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