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| Full Text | Issue Date | Title | Author(s) |  | 2012 | Monogenic mitochondrial disorders. | Koopman, W.J.H.; Willems, P.H.G.M.; Smeitink, J.A.M. |
| | 2012 | Pharmacological targeting of mitochondrial complex I deficiency: the cellular level and beyond. | Roestenberg, P.M.H.; Manjeri, G.R.; Valsecchi, F.; Smeitink, J.A.M.; Willems, P.H.G.M.; Koopman, W.J.H. |
| | 2012 | Transcriptional changes in OXPHOS complex I deficiency are related to anti-oxidant pathways and could explain the disturbed calcium homeostasis. | Voets, A.M.; Huigsloot, M.; Lindsey, P.J.; Leenders, A.M.; Koopman, W.J.H.; Willems, P.H.G.M.; Rodenburg, R.J.T.; Smeitink, J.A.M.; Smeets, H.J.M. |
| | 2012 | Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity. | Calvaruso, M.A.; Willems, P.H.; Brand, M.A. van den; Valsecchi, F.; Kruse, S.; Palmiter, R.; Smeitink, J.A.M.; Nijtmans, L.G.J. |
| | 2012 | Time-resolved quantitative analysis of CCK1 receptor-induced intracellular calcium increase. | Staljanssens, D.; Vos, W.H. De; Willems, P.H.; Camp, J. Van; Smagghe, G. |
| | 2012 | Patient-derived fibroblasts indicate oxidative stress status and may justify antioxidant therapy in OXPHOS disorders. | Voets, A.M.; Lindsey, P.J.; Vanherle, S.J.; Timmer, E.D.; Esseling, J.J.; Koopman, W.J.H.; Willems, P.H.G.M.; Schoonderwoerd, G.C.; Groote, D. De; Poll-The, B.T.; Coo, I.F.M. de; Smeets, H.J.M. |
| | 2012 | Subunit-specific Incorporation Efficiency and Kinetics in Mitochondrial Complex I Homeostasis | Dieteren, C.E.; Koopman, W.J.; Swarts, H.G.P.; Peters, J.G.; Maczuga, P.; Gemst, J. van; Masereeuw, R.; Smeitink, J.A.M.; Nijtmans, L.G.J.; Willems, P.H. |
| | 2012 | Trolox-sensitive reactive oxygen species regulate mitochondrial morphology, oxidative phosphorylation and cytosolic calcium handling in healthy cells | Distelmaier, F.; Valsecchi, F.; Forkink, M.; Emst-de Vries, S.E. van; Swarts, H.G.P.; Rodenburg, R.J.T.; Verwiel, E.T.P.; Smeitink, J.A.M.; Willems, P.H.G.M.; Koopman, W.J.H. |
| | 2012 | Modeling mitochondrial dysfunctions in the brain: from mice to men. | Breuer, M.E.; Willems, P.H.G.M.; Russel, F.G.M.; Koopman, W.J.H.; Smeitink, J.A.M. |
| | 2012 | Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts. | Valsecchi, F.; Monge, C.; Forkink, M.; Groof, A.J.C. de; Benard, G.; Rossignol, R.; Swarts, H.G.P.; Emst-de Vries, S.E. van; Rodenburg, R.J.T.; Calvaruso, M.A.; Nijtmans, L.G.J.; Heeman, B.; Roestenberg, P.M.H.; Wieringa, B.; Smeitink, J.A.M.; Koopman, W.J.H.; Willems, P.H.G.M. |
| | 2012 | A catalytic defect in mitochondrial respiratory chain complex I due to a mutation in NDUFS2 in a patient with Leigh syndrome. | Ngu, L.H.; Nijtmans, L.G.J.; Distelmaier, F.; Venselaar, H.; Emst-de Vries, S.E. van; Brand, M.A.M. van den; Stoltenborg, B.J.M.; Wintjes, L.T.; Willems, P.H.G.M.; Heuvel, L.P.W.J. van den; Smeitink, J.A.M.; Rodenburg, R.J.T. |
| | 2011 | Cell biological consequences of Leigh disease. | Distelmaier, F.; Willems, P.H.; Smeitink, J.A.; Koopman, W.J.; Mayatepek, E. |
 | 2011 | Solute diffusion is hindered in the mitochondrial matrix | Dieteren, C.E.J.; Gielen, S.C.A.M.; Nijtmans, L.G.J.; Smeitink, J.A.M.; Swarts, H.G.P.; Brock, R.; Willems, P.H.G.M.; Koopman, W.J.H. |
| | 2011 | Depletion of PINK1 affects mitochondrial metabolism, calcium homeostasis and energy maintenance | Heeman, B.; Haute, C. Van den; Aelvoet, S.A.; Valsecchi, F.; Rodenburg, R.J.T.; Reumers, V.; Debyser, Z.; Callewaert, G.; Koopman, W.J.H.; Willems, P.H.G.M.; Baekelandt, V. |
| | 2011 | G37R SOD1 mutant alters mitochondrial complex I activity, Ca(2+) uptake and ATP production. | Coussee, E.; Smet, P. De; Bogaert, E.; Elens, I.; Damme, P. van; Willems, P.H.G.M.; Koopman, W.J.H.; Bosch, L.; Callewaert, G. |
| | 2011 | Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency | Koene, S.; Willems, P.H.G.M.; Roestenberg, P.M.H.; Koopman, W.J.H.; Smeitink, J.A.M. |
| | 2011 | Quantitative glucose and ATP sensing in mammalian cells | Liemburg-Apers, D.C.; Imamura, H.; Forkink, M.; Nooteboom, M.; Swarts, H.G.P.; Brock, R.E.; Smeitink, J.A.M.; Willems, P.H.G.M.; Koopman, W.J.H. |
| | 2011 | Isolated mitochondrial complex I deficiency: explorative data analysis of patient cell parameters. | Blanchet, L.M.; Buydens, M.C.; Smeitink, J.A.M.; Willems, P.H.G.M.; Koopman, W.J.H. |
| | 2011 | Defective mitochondrial translation differently affects the live cell dynamics of complex I subunits | Dieteren, C.E.J.; Willems, P.H.G.M.; Swarts, H.G.P.; Fransen, J.; Smeitink, J.A.M.; Koopman, W.J.H.; Nijtmans, L.G.J. |
| | 2011 | Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells | Wilmer, M.J.G.; Kluijtmans, L.A.J.; Velden, T.J. van der; Willems, P.H.G.M.; Scheffer, P.G.; Masereeuw, R.; Monnens, L.A.H.; Heuvel, L.P.W.J. van den; Levtchenko, E.N. |
| | 2010 | Clinical spectrum of the pseudotumor cerebri complex in children. | Tibussek, D.; Schneider, D.T.; Meulebroecke, N. van de; Turowski, B.; Messing-Juenger, M.; Willems, P.H.G.M.; Mayatepek, E.; Distelmaier, F. |
| | 2010 | Complex I disorders: causes, mechanisms, and development of treatment strategies at the cellular level. | Valsecchi, F.; Koopman, W.J.H.; Manjeri, G.R.; Rodenburg, R.J.T.; Smeitink, J.A.M.; Willems, P.H.G.M. |
| | 2010 | Detection and manipulation of mitochondrial reactive oxygen species in mammalian cells. | Forkink, M.; Smeitink, J.A.M.; Brock, R.E.; Willems, P.H.G.M.; Koopman, W.J.H. |
| | 2010 | Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I. | Nouws, J.; Nijtmans, L.G.J.; Houten, S.M.; Brand, M. van den; Huijnen, M.A.; Venselaar, H.; Hoefs, S.J.G.; Gloerich, J.; Kronick, J.; Hutchin, T.; Willems, P.H.G.M.; Rodenburg, R.J.T.; Wanders, R.; Heuvel, L.P.W.J. van den; Smeitink, J.A.M.; Vogel, R.O. |
 | 2010 | Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation. | Koopman, W.J.H.; Nijtmans, L.G.J.; Dieteren, C.E.J.; Roestenberg, P.M.H.; Valsecchi, F.; Smeitink, J.A.M.; Willems, P.H.G.M. |
| | 2009 | The use of fluorescence correlation spectroscopy to probe mitochondrial mobility and intramatrix protein diffusion | Willems, P.H.G.M.; Swarts, H.G.P.; Hink, M.A.; Koopman, W.J.H. |
| | 2009 | C. elegans ATAD-3 is essential for mitochondrial activity and development. | Hoffmann, M.; Bellance, N.; Rossignol, R.; Koopman, W.J.H.; Willems, P.H.G.M.; Mayatepek, E.; Bossinger, O.; Distelmaier, F. |
| | 2009 | Human Golgi antiapoptotic protein modulates intracellular calcium fluxes. | Mattia, F.P. de; Gubser, C.; Dommelen, M.M.T. van; Visch, H.J.; Distelmaier, F.; Postigo, A.; Luyten, T.; Parys, J.B.; Smedt, H. de; Smith, G.L.; Willems, P.H.G.M.; Kuppeveld, F.J.M. van |
| | 2009 | Calcium and ATP handling in human NADH:Ubiquinone oxidoreductase deficiency. | Valsecchi, F.; Esseling, J.J.; Koopman, W.J.H.; Willems, P.H.G.M. |
| | 2009 | The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency. | Distelmaier, F.; Visch, H.J.; Smeitink, J.A.M.; Mayatepek, E.; Koopman, W.J.H.; Willems, P.H.G.M. |
| | 2009 | Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease. | Distelmaier, F.; Koopman, W.J.H.; Heuvel, L.P.W.J. van den; Rodenburg, R.J.T.; Mayatepek, E.; Willems, P.H.G.M.; Smeitink, J.A.M. |
| | 2009 | Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency. | Willems, P.H.G.M.; Smeitink, J.A.M.; Koopman, W.J.H. |
| | 2009 | FXYD2 and Na,K-ATPase expression in isolated human proximal tubular cells: disturbed upregulation on renal hypomagnesemia? | Cairo, E.R.; Swarts, H.G.P.; Wilmer, M.J.G.; Willems, P.H.G.M.; Levtchenko, E.N.; Pont, J.J.H.H.M. de; Koenderink, J.B. |
| | 2009 | The non-gastric H,K-ATPase as a tool to study the ouabain-binding site in Na,K-ATPase. | Pont, J.J.H.H.M. de; Swarts, H.G.P.; Karawajczyk, A.; Schaftenaar, G.; Willems, P.H.G.M.; Koenderink, J.B. |
| | 2009 | Contiguous gene deletion of ELOVL7, ERCC8 and NDUFAF2 in a patient with a fatal multisystem disorder. | Janssen, R.J.R.J.; Distelmaier, F.; Smeets, R.J.P.; Wijnhoven, T.J.M.; Ostergaard, E.; Jaspers, N.G.; Raams, A.; Kemp, S.; Rodenburg, R.J.T.; Willems, P.H.G.M.; Heuvel, L.P.W.J. van den; Smeitink, J.A.M.; Nijtmans, L.G.J. |
| | 2009 | Baculovirus complementation restores a novel NDUFAF2 mutation causing complex I deficiency. | Hoefs, S.J.G.; Dieteren, C.E.J.; Rodenburg, R.J.T.; Naess, K.; Bruhn, H.; Wibom, R.; Wagena, E.; Willems, P.H.G.M.; Smeitink, J.A.M.; Nijtmans, L.G.J.; Heuvel, L.P.W.J. van den |
| | 2009 | Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease. | Saada, A.; Vogel, R.O.; Hoefs, S.J.G.; Brand, M.A.M. van den; Wessels, H.J.; Willems, P.H.G.M.; Venselaar, H.; Shaag, A.; Barghuti, F.; Reish, O.; Shohat, M.; Huijnen, M.A.; Smeitink, J.A.M.; Heuvel, L.P.W.J. van den; Nijtmans, L.G.J. |
| | 2009 | Parenteral medium-chain triglyceride-induced neutrophil activation is not mediated by a Pertussis Toxin sensitive receptor. | Versleijen, M.W.J.; Esterik, J.C. van; Schaap-Roelofs, H.M.J.; Emst-de Vries, S.E. van; Willems, P.H.G.M.; Wanten, G.J.A. |
| | 2008 | Impaired routing of wild type FXYD2 after oligomerisation with FXYD2-G41R might explain the dominant nature of renal hypomagnesemia. | Cairo, E.R.; Friedrich, T.; Swarts, H.G.P.; Knoers, N.V.A.M.; Bindels, R.J.M.; Monnens, L.A.; Willems, P.H.G.M.; Pont, J.J.H.H.M. de; Koenderink, J.B. |
| | 2008 | Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency. | Willems, P.H.G.M.; Valsecchi, F.; Distelmaier, F.; Verkaart, S.; Visch, H.J.; Smeitink, J.A.M.; Koopman, W.J.H. |
| | 2008 | Life cell quantification of mitochondrial membrane potential at the single organelle level. | Distelmaier, F.; Koopman, W.J.; Testa, E.R.; Jong, AS de; Swarts, H.G.P.; Mayatepek, E.; Smeitink, J.A.M.; Willems, P.H.G.M. |
| | 2008 | Computer-assisted live cell analysis of mitochondrial membrane potential, morphology and calcium handling. | Koopman, W.J.H.; Distelmaier, F.; Esseling, J.J.; Smeitink, J.A.M.; Willems, P.H.G.M. |
| | 2008 | Subunits of mitochondrial complex I exist as part of matrix- and membrane-associated subcomplexes in living cells. | Dieteren, C.E.J.; Willems, P.H.G.M.; Vogel, R.O.; Swarts, H.G.P.; Fransen, J.; Roepman, R.; Crienen, G.; Smeitink, J.A.M.; Nijtmans, L.G.J.; Koopman, W.J.H. |
| | 2008 | NDUFA2 complex I mutation leads to Leigh disease. | Hoefs, S.J.; Dieteren, C.E.; Distelmaier, F.; Janssen, R.J.; Epplen, A.; Swarts, H.G.P.; Forkink, M.; Rodenburg, R.J.; Nijtmans, L.G.J.; Willems, P.H.G.M.; Smeitink, J.A.M.; Heuvel, L.P.v.d. |
| | 2008 | Functional analysis of picornavirus 2B proteins: effects on calcium homeostasis and intracellular protein trafficking. | Jong, A.S. de; Mattia, F.P. de; Dommelen, M.M.T.; Lanke, K.H.W.; Melchers, W.J.G.; Willems, P.H.G.M.; Kuppeveld, F.J.M. van |
| | 2008 | Mitochondrial processes are impaired in hereditary inclusion body myopathy. | Eisenberg, I.; Novershtern, N.; Itzhaki, Z.; Becker-Cohen, M.; Sadeh, M.; Willems, P.H.G.M.; Friedman, N.; Koopman, W.J.H.; Mitrani-Rosenbaum, S. |
| | 2008 | Mitochondrial function and morphology are impaired in parkin-mutant fibroblasts. | Mortiboys, H.; Thomas, K.J.; Koopman, W.J.H.; Klaffke, S.; Abou-Sleiman, P.; Olpin, S.; Wood, N.W.; Willems, P.H.G.M.; Smeitink, J.A.M.; Cookson, M.R.; Bandmann, O. |
| | 2008 | Inherited complex I deficiency is associated with faster protein diffusion in the matrix of moving mitochondria | Koopman, W.J.H.; Distelmaier, F.; Hink, M.A.; Verkaart, S.; Wijers, M.; Fransen, J.; Smeitink, J.A.M.; Willems, P.H.G.M. |
| | 2008 | Immune function and leukocyte sequestration under the influence of parenteral lipid emulsions in healthy humans: a placebo-controlled crossover study. | Versleijen, M.W.J.; Oyen, W.J.G.; Roelofs, H.M.J.; Emst-de Vries, S.E. van; Willems, P.H.G.M.; Jansen, J.B.M.J.; Wanten, G.J.A. |
| | 2008 | Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment. | Koopman, W.J.H.; Verkaart, S.; Emst-de Vries, S.E. van; Grefte, S.; Smeitink, J.A.M.; Nijtmans, L.G.J.; Willems, P.H.G.M. |
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