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Items: 1 to 50 of 218

1.

Homozygous damaging SOD2 variant causes lethal neonatal dilated cardiomyopathy.

Almomani R, Herkert JC, Posafalvi A, Post JG, Boven LG, van der Zwaag PA, Willems PHGM, van Veen-Hof IH, Verhagen JMA, Wessels MW, Nikkels PGJ, Wintjes LT, van den Berg MP, Sinke RJ, Rodenburg RJ, Niezen-Koning KE, van Tintelen JP, Jongbloed JDH.

J Med Genet. 2019 Sep 7. pii: jmedgenet-2019-106330. doi: 10.1136/jmedgenet-2019-106330. [Epub ahead of print]

PMID:
31494578
2.

Octa-arginine boosts the penetration of elastin-like polypeptide nanoparticles in 3D cancer models.

van Oppen LMPE, Pille J, Stuut C, van Stevendaal M, van der Vorm LN, Smeitink JAM, Koopman WJH, Willems PHGM, van Hest JCM, Brock R.

Eur J Pharm Biopharm. 2019 Apr;137:175-184. doi: 10.1016/j.ejpb.2019.02.010. Epub 2019 Feb 15.

PMID:
30776413
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 14;9(11):1135. doi: 10.1038/s41419-018-1179-4.

4.

Mitochondrial Morphofunction in Mammalian Cells.

Bulthuis EP, Adjobo-Hermans MJW, Willems PHGM, Koopman WJH.

Antioxid Redox Signal. 2019 Jun 20;30(18):2066-2109. doi: 10.1089/ars.2018.7534. Epub 2018 Nov 29.

5.

Corrigendum to "Mitigation of NADH:ubiquinone oxidoreductase deficiency by chronic Trolox treatment" [Biochimica et Biophysica Acta 1777/7-8 (2008) 853-859].

Koopman WJH, Verkaart S, van Emst-de Vries SE, Grefte S, Smeitink JAM, Nijtmans LGJ, Willems PHGM.

Biochim Biophys Acta Bioenerg. 2018 Dec;1859(12):1328. doi: 10.1016/j.bbabio.2018.06.003. Epub 2018 Jun 6. No abstract available.

PMID:
29883590
6.

Improvements in fitness are not obligatory for exercise training-induced improvements in CV risk factors.

Hartman YAW, Hopman MTE, Schreuder TH, Verheggen RJHM, Scholten RR, Oudegeest-Sander MH, Poelkens F, Maiorana AJ, Naylor LH, Willems PH, Tack CJ, Thijssen DHJ, Green DJ.

Physiol Rep. 2018 Feb;6(4). doi: 10.14814/phy2.13595.

7.

Extracellular acidification induces ROS- and mPTP-mediated death in HEK293 cells.

Teixeira J, Basit F, Swarts HG, Forkink M, Oliveira PJ, Willems PHGM, Koopman WJH.

Redox Biol. 2018 May;15:394-404. doi: 10.1016/j.redox.2017.12.018. Epub 2017 Dec 30.

8.

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 24;8(1):150. doi: 10.1186/s13287-017-0601-7.

9.

Controlling T-Cell Activation with Synthetic Dendritic Cells Using the Multivalency Effect.

Hammink R, Mandal S, Eggermont LJ, Nooteboom M, Willems PH, Tel J, Rowan AE, Figdor CG, Blank KG.

ACS Omega. 2017 Mar 31;2(3):937-945. doi: 10.1021/acsomega.6b00436. Epub 2017 Mar 16.

10.

Acute stimulation of glucose influx upon mitoenergetic dysfunction requires LKB1, AMPK, Sirt2 and mTOR-RAPTOR.

Liemburg-Apers DC, Wagenaars JA, Smeitink JA, Willems PH, Koopman WJ.

J Cell Sci. 2016 Dec 1;129(23):4411-4423. Epub 2016 Oct 28.

11.

Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy.

Iannetti EF, Smeitink JA, Beyrath J, Willems PH, Koopman WJ.

Nat Protoc. 2016 Sep;11(9):1693-710. doi: 10.1038/nprot.2016.094. Epub 2016 Aug 18.

PMID:
27560174
12.

Integrated High-Content Quantification of Intracellular ROS Levels and Mitochondrial Morphofunction.

Sieprath T, Corne TD, Willems PH, Koopman WJ, De Vos WH.

Adv Anat Embryol Cell Biol. 2016;219:149-77. doi: 10.1007/978-3-319-28549-8_6. Review.

PMID:
27207366
13.

Mitochondrial disorders in children: toward development of small-molecule treatment strategies.

Koopman WJ, Beyrath J, Fung CW, Koene S, Rodenburg RJ, Willems PH, Smeitink JA.

EMBO Mol Med. 2016 Apr 1;8(4):311-27. doi: 10.15252/emmm.201506131. Review.

14.

Broad defects in the energy metabolism of leukocytes underlie immunoparalysis in sepsis.

Cheng SC, Scicluna BP, Arts RJ, Gresnigt MS, Lachmandas E, Giamarellos-Bourboulis EJ, Kox M, Manjeri GR, Wagenaars JA, Cremer OL, Leentjens J, van der Meer AJ, van de Veerdonk FL, Bonten MJ, Schultz MJ, Willems PH, Pickkers P, Joosten LA, van der Poll T, Netea MG.

Nat Immunol. 2016 Apr;17(4):406-13. doi: 10.1038/ni.3398. Epub 2016 Mar 7.

PMID:
26950237
15.

Complex I and complex III inhibition specifically increase cytosolic hydrogen peroxide levels without inducing oxidative stress in HEK293 cells.

Forkink M, Basit F, Teixeira J, Swarts HG, Koopman WJH, Willems PHGM.

Redox Biol. 2015 Dec;6:607-616. doi: 10.1016/j.redox.2015.09.003. Epub 2015 Oct 23.

16.

Targeting mitochondrial complex I using BAY 87-2243 reduces melanoma tumor growth.

Schöckel L, Glasauer A, Basit F, Bitschar K, Truong H, Erdmann G, Algire C, Hägebarth A, Willems PH, Kopitz C, Koopman WJ, Héroult M.

Cancer Metab. 2015 Oct 20;3:11. doi: 10.1186/s40170-015-0138-0. eCollection 2015.

17.

Mitoenergetic Dysfunction Triggers a Rapid Compensatory Increase in Steady-State Glucose Flux.

Liemburg-Apers DC, Schirris TJ, Russel FG, Willems PH, Koopman WJ.

Biophys J. 2015 Oct 6;109(7):1372-86. doi: 10.1016/j.bpj.2015.08.002.

18.

Mitochondrial ADP/ATP exchange inhibition: a novel off-target mechanism underlying ibipinabant-induced myotoxicity.

Schirris TJ, Ritschel T, Herma Renkema G, Willems PH, Smeitink JA, Russel FG.

Sci Rep. 2015 Sep 29;5:14533. doi: 10.1038/srep14533.

19.

Statin-Induced Myopathy Is Associated with Mitochondrial Complex III Inhibition.

Schirris 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.

Cell Metab. 2015 Sep 1;22(3):399-407. doi: 10.1016/j.cmet.2015.08.002.

20.

Increased mitochondrial ATP production capacity in brain of healthy mice and a mouse model of isolated complex I deficiency after isoflurane anesthesia.

Manjeri GR, Rodenburg RJ, Blanchet L, Roelofs S, Nijtmans LG, Smeitink JA, Driessen JJ, Koopman WJ, Willems PH.

J Inherit Metab Dis. 2016 Jan;39(1):59-65. doi: 10.1007/s10545-015-9885-x. Epub 2015 Aug 27.

21.

Redox Homeostasis and Mitochondrial Dynamics.

Willems PH, Rossignol R, Dieteren CE, Murphy MP, Koopman WJ.

Cell Metab. 2015 Aug 4;22(2):207-18. doi: 10.1016/j.cmet.2015.06.006. Epub 2015 Jul 9. Review.

22.

Interactions between mitochondrial reactive oxygen species and cellular glucose metabolism.

Liemburg-Apers DC, Willems PH, Koopman WJ, Grefte S.

Arch Toxicol. 2015 Aug;89(8):1209-26. doi: 10.1007/s00204-015-1520-y. Epub 2015 Jun 6. Review.

23.

Sustained accumulation of prelamin A and depletion of lamin A/C both cause oxidative stress and mitochondrial dysfunction but induce different cell fates.

Sieprath T, Corne TD, Nooteboom M, Grootaert C, Rajkovic A, Buysschaert B, Robijns J, Broers JL, Ramaekers FC, Koopman WJ, Willems PH, De Vos WH.

Nucleus. 2015;6(3):236-46. doi: 10.1080/19491034.2015.1050568. Epub 2015 May 21.

24.

Rotenone inhibits primary murine myotube formation via Raf-1 and ROCK2.

Grefte S, Wagenaars JA, Jansen R, Willems PH, Koopman WJ.

Biochim Biophys Acta. 2015 Jul;1853(7):1606-14. doi: 10.1016/j.bbamcr.2015.03.010. Epub 2015 Mar 28.

25.

PKC-mediated inhibitory feedback of the cholecystokinin 1 receptor controls the shape of oscillatory Ca²⁺ signals.

Willems PH, Pahle J, Stalpers XL, Mugahid D, Nikolaew A, Koopman WJ, Kummer U.

FEBS J. 2015 Jun;282(11):2187-201. doi: 10.1111/febs.13267. Epub 2015 Apr 13.

26.

Skeletal muscle mitochondria of NDUFS4-/- mice display normal maximal pyruvate oxidation and ATP production.

Alam MT, Manjeri GR, Rodenburg RJ, Smeitink JA, Notebaart RA, Huynen M, Willems PH, Koopman WJ.

Biochim Biophys Acta. 2015 Jun-Jul;1847(6-7):526-33. doi: 10.1016/j.bbabio.2015.02.006. Epub 2015 Feb 14.

27.

Toward high-content screening of mitochondrial morphology and membrane potential in living cells.

Iannetti EF, Willems PH, Pellegrini M, Beyrath J, Smeitink JA, Blanchet L, Koopman WJ.

Int J Biochem Cell Biol. 2015 Jun;63:66-70. doi: 10.1016/j.biocel.2015.01.020. Epub 2015 Feb 8. Review.

PMID:
25668473
28.

Live-cell assessment of mitochondrial reactive oxygen species using dihydroethidine.

Forkink M, Willems PH, Koopman WJ, Grefte S.

Methods Mol Biol. 2015;1264:161-9. doi: 10.1007/978-1-4939-2257-4_15.

PMID:
25631012
29.

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 26;5:8035. doi: 10.1038/srep08035.

30.

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.

Biochim Biophys Acta. 2015 Mar;1852(3):529-40. doi: 10.1016/j.bbadis.2014.12.012. Epub 2014 Dec 20.

31.

Photo-induction and automated quantification of reversible mitochondrial permeability transition pore opening in primary mouse myotubes.

Blanchet L, Grefte S, Smeitink JA, Willems PH, Koopman WJ.

PLoS One. 2014 Nov 25;9(11):e114090. doi: 10.1371/journal.pone.0114090. eCollection 2014.

32.

Automated quantification and integrative analysis of 2D and 3D mitochondrial shape and network properties.

Nikolaisen J, Nilsson LI, Pettersen IK, Willems PH, Lorens JB, Koopman WJ, Tronstad KJ.

PLoS One. 2014 Jul 2;9(7):e101365. doi: 10.1371/journal.pone.0101365. eCollection 2014.

33.

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.

Biochim Biophys Acta. 2014 Aug;1837(8):1247-56. doi: 10.1016/j.bbabio.2014.04.008. Epub 2014 Apr 24.

34.

Function and regulation of the Na+-Ca2+ exchanger NCX3 splice variants in brain and skeletal muscle.

Michel LY, Verkaart S, Koopman WJ, Willems PH, Hoenderop JG, Bindels RJ.

J Biol Chem. 2014 Apr 18;289(16):11293-303. doi: 10.1074/jbc.M113.529388. Epub 2014 Mar 10.

35.

Regulation and quantification of cellular mitochondrial morphology and content.

Tronstad KJ, Nooteboom M, Nilsson LI, Nikolaisen J, Sokolewicz M, Grefte S, Pettersen IK, Dyrstad S, Hoel F, Willems PH, Koopman WJ.

Curr Pharm Des. 2014;20(35):5634-52. Review.

PMID:
24606803
36.

Isoflurane anesthetic hypersensitivity and progressive respiratory depression in a mouse model with isolated mitochondrial complex I deficiency.

Roelofs S, Manjeri GR, Willems PH, Scheffer GJ, Smeitink JA, Driessen JJ.

J Anesth. 2014 Dec;28(6):807-14. doi: 10.1007/s00540-014-1791-0. Epub 2014 Feb 13.

PMID:
24522811
37.

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. doi: 10.1002/iub.1127. Epub 2013 Feb 3. Review.

38.

Primary fibroblasts of NDUFS4(-/-) mice display increased ROS levels and aberrant mitochondrial morphology.

Valsecchi F, Grefte S, Roestenberg P, Joosten-Wagenaars J, Smeitink JA, Willems PH, Koopman WJ.

Mitochondrion. 2013 Sep;13(5):436-43. doi: 10.1016/j.mito.2012.12.001. Epub 2012 Dec 9.

PMID:
23234723
39.

OXPHOS mutations and neurodegeneration.

Koopman WJ, Distelmaier F, Smeitink JA, Willems PH.

EMBO J. 2013 Jan 9;32(1):9-29. doi: 10.1038/emboj.2012.300. Epub 2012 Nov 13. Review.

40.

A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia.

Szklarczyk R, Wanschers BF, Nijtmans LG, Rodenburg RJ, Zschocke J, Dikow N, van den Brand MA, Hendriks-Franssen MG, Gilissen C, Veltman JA, Nooteboom M, Koopman WJ, Willems PH, Smeitink JA, Huynen MA, van den Heuvel LP.

Hum Mol Genet. 2013 Feb 15;22(4):656-67. doi: 10.1093/hmg/dds473. Epub 2012 Nov 2.

PMID:
23125284
41.

Subunit-specific incorporation efficiency and kinetics in mitochondrial complex I homeostasis.

Dieteren CE, Koopman WJ, Swarts HG, Peters JG, Maczuga P, van Gemst JJ, Masereeuw R, Smeitink JA, Nijtmans LG, Willems PH.

J Biol Chem. 2012 Dec 7;287(50):41851-60. doi: 10.1074/jbc.M112.391151. Epub 2012 Oct 4.

42.

Patient-derived fibroblasts indicate oxidative stress status and may justify antioxidant therapy in OXPHOS disorders.

Voets AM, Lindsey PJ, Vanherle SJ, Timmer ED, Esseling JJ, Koopman WJ, Willems PH, Schoonderwoerd GC, De Groote D, Poll-The BT, de Coo IF, Smeets HJ.

Biochim Biophys Acta. 2012 Nov;1817(11):1971-8. doi: 10.1016/j.bbabio.2012.07.001. Epub 2012 Jul 13.

43.

Trolox-sensitive reactive oxygen species regulate mitochondrial morphology, oxidative phosphorylation and cytosolic calcium handling in healthy cells.

Distelmaier F, Valsecchi F, Forkink M, van Emst-de Vries S, Swarts HG, Rodenburg RJ, Verwiel ET, Smeitink JA, Willems PH, Koopman WJ.

Antioxid Redox Signal. 2012 Dec 15;17(12):1657-69. doi: 10.1089/ars.2011.4294. Epub 2012 Jun 13.

44.

Monogenic mitochondrial disorders.

Koopman WJ, Willems PH, Smeitink JA.

N Engl J Med. 2012 Mar 22;366(12):1132-41. doi: 10.1056/NEJMra1012478. Review. No abstract available.

PMID:
22435372
45.

Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts.

Valsecchi F, Monge C, Forkink M, de Groof AJ, Benard G, Rossignol R, Swarts HG, van Emst-de Vries SE, Rodenburg RJ, Calvaruso MA, Nijtmans LG, Heeman B, Roestenberg P, Wieringa B, Smeitink JA, Koopman WJ, Willems PH.

Biochim Biophys Acta. 2012 Oct;1817(10):1925-36. doi: 10.1016/j.bbabio.2012.03.006. Epub 2012 Mar 11.

46.

The role of mitochondrial OXPHOS dysfunction in the development of neurologic diseases.

Breuer ME, Koopman WJ, Koene S, Nooteboom M, Rodenburg RJ, Willems PH, Smeitink JA.

Neurobiol Dis. 2013 Mar;51:27-34. doi: 10.1016/j.nbd.2012.03.007. Epub 2012 Mar 9. Review.

PMID:
22426394
47.

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 1;17(36):4023-33. Review.

PMID:
22188452
48.

A catalytic defect in mitochondrial respiratory chain complex I due to a mutation in NDUFS2 in a patient with Leigh syndrome.

Ngu LH, Nijtmans LG, Distelmaier F, Venselaar H, van Emst-de Vries SE, van den Brand MA, Stoltenborg BJ, Wintjes LT, Willems PH, van den Heuvel LP, Smeitink JA, Rodenburg RJ.

Biochim Biophys Acta. 2012 Feb;1822(2):168-75. doi: 10.1016/j.bbadis.2011.10.012. Epub 2011 Oct 20.

49.

Transcriptional changes in OXPHOS complex I deficiency are related to anti-oxidant pathways and could explain the disturbed calcium homeostasis.

Voets AM, Huigsloot M, Lindsey PJ, Leenders AM, Koopman WJ, Willems PH, Rodenburg RJ, Smeitink JA, Smeets HJ.

Biochim Biophys Acta. 2012 Jul;1822(7):1161-8. doi: 10.1016/j.bbadis.2011.10.009. Epub 2011 Oct 17.

50.

Defective mitochondrial translation differently affects the live cell dynamics of complex I subunits.

Dieteren CE, Willems PH, Swarts HG, Fransen J, Smeitink JA, Koopman WJ, Nijtmans LG.

Biochim Biophys Acta. 2011 Dec;1807(12):1624-33. doi: 10.1016/j.bbabio.2011.09.013. Epub 2011 Sep 24.

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