Leo G. Nijtmans

Overview

Best Publications

• Defective Remodeling of Cardiolipin and Phosphatidylglycerol in Barth Syndrome

  Peter Vreken;Fredoen Valianpour;Leo G. Nijtmans;Les A. Grivell

  503
  Citations

• Mammalian Mitochondrial Complex I: Biogenesis, Regulation, and Reactive Oxygen Species Generation

  Werner J.H. Koopman;Leo G.J. Nijtmans;Cindy E.J. Dieteren;Peggy Roestenberg

  470
  Citations

• Blue Native electrophoresis to study mitochondrial and other protein complexes.

  Leo G J Nijtmans;Nadine S Henderson;Ian J Holt

  433
  Citations

• The Assembly Pathway of Mitochondrial Respiratory Chain Complex I.

  Sergio Guerrero-Castillo;Sergio Guerrero-Castillo;Fabian Baertling;Fabian Baertling;Daniel Kownatzki;Hans J. Wessels

  407
  Citations

• Mitochondrial complex I: structure, function and pathology.

  Rolf J. R. J. Janssen;Leo G. Nijtmans;Lambert P. van den Heuvel;Jan A. M. Smeitink

  338
  Citations

• The Mitochondrial Prohibitin Complex Is Essential for Embryonic Viability and Germline Function in Caenorhabditis elegans

  Marta Artal Sanz;William Y. Tsang;Esther M. Willems;Les A. Grivell

  289
  Citations

• Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly.

  Rutger O. Vogel;Rolf J.R.J. Janssen;Mariël A.M. van den Brand;Cindy E.J. Dieteren

  257
  Citations

• Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency

  Cristina Ugalde;Rolf J.R.J. Janssen;Lambert P. van den Heuvel;Jan A.M. Smeitink

  249
  Citations

• Mutations in the phospholipid remodeling gene SERAC1 impair mitochondrial function and intracellular cholesterol trafficking and cause dystonia and deafness

  Saskia B Wortmann;Frédéric M Vaz;Thatjana Gardeitchik;Lisenka E L M Vissers

  244
  Citations

• Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies

  Cristina Ugalde;Rutger Vogel;Richard Huijbens;Bert van den Heuvel

  230
  Citations

• A structure for the yeast prohibitin complex: Structure prediction and evidence from chemical crosslinking and mass spectrometry.

  Jaap W. Back;Marta Artal Sanz;Luitzen de Jong;Leo J. de Koning

  227
  Citations

• Acyl-CoA Dehydrogenase 9 Is Required for the Biogenesis of Oxidative Phosphorylation Complex I

  Jessica Nouws;Leo Nijtmans;Sander M. Houten;Mariël van den Brand

  217
  Citations

• Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease.

  Ann Saada;Rutger O. Vogel;Saskia J. Hoefs;Mariël A. van den Brand

  213
  Citations

• Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunits.

  Rutger O. Vogel;Cindy E.J. Dieteren;Lambert P. W.J. van den Heuvel;Peter H. G.M. Willems

  203
  Citations

• TEFM (c17orf42) is necessary for transcription of human mtDNA

  Michal Minczuk;Jiuya He;Anna M. Duch;Thijs J. Ettema

  202
  Citations

• Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins

  Marina A. Schwab;Sven W. Sauer;Jürgen G. Okun;Leo G. J. Nijtmans

  195
  Citations

• Human mitochondrial complex I assembly: a dynamic and versatile process.

  Rutger O. Vogel;Jan A.M. Smeitink;Leo G.J. Nijtmans

  194
  Citations

• Superoxide production is inversely related to complex I activity in inherited complex I deficiency

  Sjoerd Verkaart;Werner J.H. Koopman;Sjenet E. van Emst-de Vries;Leo G.J. Nijtmans

  187
  Citations

• Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.

  Rosa Pello;Miguel A. Martín;Valerio Carelli;Leo G. Nijtmans

  185
  Citations

• Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?

  Werner J. H. Koopman;Sjoerd Verkaart;Henk Jan Visch;Sjenet van Emst-de Vries

  179
  Citations