Leo G J Nijtmans

Summary

Affiliation: University Medical Centre Nijmegen
Country: The Netherlands

Publications

  1. pmc Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly
    Rutger O Vogel
    Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen 6500 HB, The Netherlands
    Genes Dev 21:615-24. 2007
  2. ncbi request reprint Blue Native electrophoresis to study mitochondrial and other protein complexes
    Leo G J Nijtmans
    Nijmegen Centre for Mitochondrial Disorders, University Medical Centre Nijmegen, Geert Grooteplein 10, PO Box 9101, 6500 HB Nijmegen, The Netherlands
    Methods 26:327-34. 2002
  3. doi request reprint Defective mitochondrial translation differently affects the live cell dynamics of complex I subunits
    Cindy E J Dieteren
    Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB, Nijmegen, The Netherlands
    Biochim Biophys Acta 1807:1624-33. 2011
  4. pmc Subunits of mitochondrial complex I exist as part of matrix- and membrane-associated subcomplexes in living cells
    Cindy E J Dieteren
    Department of Biochemistry, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    J Biol Chem 283:34753-61. 2008
  5. ncbi request reprint Investigation of the complex I assembly chaperones B17.2L and NDUFAF1 in a cohort of CI deficient patients
    Rutger O Vogel
    Nijmegen Centre for Mitochondrial Disorders, Department of Paediatrics, Radboud University Nijmegen Medical Centre, 6500 HB, Nijmegen, The Netherlands
    Mol Genet Metab 91:176-82. 2007
  6. pmc Subunit-specific incorporation efficiency and kinetics in mitochondrial complex I homeostasis
    Cindy E J Dieteren
    Department of Biochemistry, Radboud University Nijmegen Medical Centre, 6500 HB Nijmegen, The Netherlands
    J Biol Chem 287:41851-60. 2012
  7. pmc Solute diffusion is hindered in the mitochondrial matrix
    Cindy E J Dieteren
    Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, PO Box 9101, NL 6500 HB Nijmegen, The Netherlands
    Proc Natl Acad Sci U S A 108:8657-62. 2011
  8. ncbi request reprint Human mitochondrial complex I assembly: a dynamic and versatile process
    Rutger O Vogel
    Nijmegen Centre for Mitochondrial Disorders, Department of Pediatrics, Radboud University Nijmegen Medical Centre, Geert Grooteplein 10, 6500 HB Nijmegen, The Netherlands
    Biochim Biophys Acta 1767:1215-27. 2007
  9. ncbi request reprint Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunits
    Rutger O Vogel
    Nijmegen Centre for Mitochondrial Disorders, Department of Paediatrics, Radboud University Nijmegen Medical Centre, 6500 HB Nijmegen, The Netherlands
    J Biol Chem 282:7582-90. 2007
  10. doi request reprint Chapter 7 Tracing human mitochondrial complex I assembly by use of GFP-tagged subunits
    Cindy E J Dieteren
    Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Methods Enzymol 456:133-51. 2009

Collaborators

Detail Information

Publications21

  1. pmc Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly
    Rutger O Vogel
    Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen 6500 HB, The Netherlands
    Genes Dev 21:615-24. 2007
    ..These findings support a function for Ecsit in the assembly or stability of mitochondrial complex I, possibly linking assembly of oxidative phosphorylation complexes to inflammatory response and embryonic development...
  2. ncbi request reprint Blue Native electrophoresis to study mitochondrial and other protein complexes
    Leo G J Nijtmans
    Nijmegen Centre for Mitochondrial Disorders, University Medical Centre Nijmegen, Geert Grooteplein 10, PO Box 9101, 6500 HB Nijmegen, The Netherlands
    Methods 26:327-34. 2002
    ..Finally, new ways of identifying protein spots in Blue Native gels using mass spectrometry are briefly discussed...
  3. doi request reprint Defective mitochondrial translation differently affects the live cell dynamics of complex I subunits
    Cindy E J Dieteren
    Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB, Nijmegen, The Netherlands
    Biochim Biophys Acta 1807:1624-33. 2011
    ..Our results show that the protein interactions of CI subunits and assembly factors are differently altered when mitochondrial translation is defective...
  4. pmc Subunits of mitochondrial complex I exist as part of matrix- and membrane-associated subcomplexes in living cells
    Cindy E J Dieteren
    Department of Biochemistry, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    J Biol Chem 283:34753-61. 2008
    ..Our results provide first time evidence for the existence of CI subassemblies in mitochondria of living cells...
  5. ncbi request reprint Investigation of the complex I assembly chaperones B17.2L and NDUFAF1 in a cohort of CI deficient patients
    Rutger O Vogel
    Nijmegen Centre for Mitochondrial Disorders, Department of Paediatrics, Radboud University Nijmegen Medical Centre, 6500 HB, Nijmegen, The Netherlands
    Mol Genet Metab 91:176-82. 2007
    ..These findings illustrate the difference in mechanism between B17.2L and NDUFAF1 and suggest that the involvement of NDUFAF1 in the assembly process could be indirect rather than direct via the binding to assembly intermediates...
  6. pmc Subunit-specific incorporation efficiency and kinetics in mitochondrial complex I homeostasis
    Cindy E J Dieteren
    Department of Biochemistry, Radboud University Nijmegen Medical Centre, 6500 HB Nijmegen, The Netherlands
    J Biol Chem 287:41851-60. 2012
    ..In conclusion, the results presented demonstrate that newly formed nDNA-encoded CI subunits rapidly incorporate into holo-CI in a subunit-specific manner...
  7. pmc Solute diffusion is hindered in the mitochondrial matrix
    Cindy E J Dieteren
    Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, PO Box 9101, NL 6500 HB Nijmegen, The Netherlands
    Proc Natl Acad Sci U S A 108:8657-62. 2011
    ....
  8. ncbi request reprint Human mitochondrial complex I assembly: a dynamic and versatile process
    Rutger O Vogel
    Nijmegen Centre for Mitochondrial Disorders, Department of Pediatrics, Radboud University Nijmegen Medical Centre, Geert Grooteplein 10, 6500 HB Nijmegen, The Netherlands
    Biochim Biophys Acta 1767:1215-27. 2007
    ..This is where chaperone proteins come into play, some of which may relate complex I assembly to processes such as apoptosis and even immunity...
  9. ncbi request reprint Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunits
    Rutger O Vogel
    Nijmegen Centre for Mitochondrial Disorders, Department of Paediatrics, Radboud University Nijmegen Medical Centre, 6500 HB Nijmegen, The Netherlands
    J Biol Chem 282:7582-90. 2007
    ..Composition analysis showed that this event was accompanied by the incorporation of at least one mitochondrial DNA-encoded subunit, thereby revealing the first entry point of these subunits...
  10. doi request reprint Chapter 7 Tracing human mitochondrial complex I assembly by use of GFP-tagged subunits
    Cindy E J Dieteren
    Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Methods Enzymol 456:133-51. 2009
    ....
  11. ncbi request reprint Mutated ND2 impairs mitochondrial complex I assembly and leads to Leigh syndrome
    Cristina Ugalde
    Nijmegen Center for Mitochondrial Disorders, Department of Pediatrics, Radboud University Nijmegen Medical Center, Geert Grooteplein 10, 6500 HB Nijmegen, The Netherlands
    Mol Genet Metab 90:10-4. 2007
    ....
  12. ncbi request reprint Cytochrome c oxidase biogenesis in a patient with a mutation in COX10 gene
    Marieke J H Coenen
    Department of Paediatrics, Nijmegen Centre for Mitochondrial Disorders, University Medical Centre Nijmegen, Nijmegen, The Netherlands
    Ann Neurol 56:560-4. 2004
    ..Overexpression of the COX10 protein in the patient's fibroblasts proved that the detected mutation was indeed the disease cause...
  13. ncbi request reprint Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency
    Cristina Ugalde
    Nijmegen Center for Mitochondrial Disorders, Department of Pediatrics, University Medical Center, Nijmegen, The Netherlands
    Hum Mol Genet 13:659-67. 2004
    ..This will help us to point more selectively to candidate genes for pathogenic mutations that could lead to an isolated complex I defect...
  14. ncbi request reprint Cell biological consequences of mitochondrial NADH: ubiquinone oxidoreductase deficiency
    Jan A M Smeitink
    Department of Pediatrics, Nijmegen Center for Mitochondrial Disorders, University Medical Center Nijmegen, The Netherlands
    Curr Neurovasc Res 1:29-40. 2004
    ....
  15. pmc A structure for the yeast prohibitin complex: Structure prediction and evidence from chemical crosslinking and mass spectrometry
    Jaap W Back
    Swammerdam Institute for Life Sciences SILS Mass Spectrometry Group, University of Amsterdam, Nieuwe Achtergracht 166, The Netherlands
    Protein Sci 11:2471-8. 2002
    ..Each unit contains a putative transmembrane helix in PHB2. Taken together with the unit building block we postulate a circular palisade-like arrangement of the building blocks projecting into the intermembrane space...
  16. ncbi request reprint The mitochondrial prohibitin complex is essential for embryonic viability and germline function in Caenorhabditis elegans
    Marta Artal-Sanz
    Swammerdam Institute for Life Sciences, Section for Molecular Biology, University of Amsterdam, Kruislaan 318, Amsterdam 1098 SM, The Netherlands
    J Biol Chem 278:32091-9. 2003
    ....
  17. ncbi request reprint Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment
    Werner J H Koopman
    Department of Biochemistry, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, The Netherlands
    Biochim Biophys Acta 1777:853-9. 2008
    ..However, such treatment may only be beneficial to patients with a relatively small reduction in intrinsic catalytic defect of the complex...
  18. ncbi request reprint Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?
    Werner J H Koopman
    Department of Membrane Biochemistry, Radboud University Nijmegen Medical Centre, P O Box 9101, NL 6500 HB Nijmegen, The Netherlands
    Am J Physiol Cell Physiol 293:C22-9. 2007
    ....
  19. pmc Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins
    Marina A Schwab
    Department of General Pediatrics, Division of Inborn Metabolic Diseases, University Children s Hospital Heidelberg, Im Neuenheimer Feld 150, D 69120 Heidelberg, Germany
    Biochem J 398:107-12. 2006
    ....
  20. ncbi request reprint Human mitochondrial complex I assembly is mediated by NDUFAF1
    Rutger O Vogel
    Department of Paediatrics, Radboud University Nijmegen Medical Centre, Netherlands
    FEBS J 272:5317-26. 2005
    ..Based on these data, we propose that NDUFAF1 is an important protein for the assembly/stability of complex I...
  21. ncbi request reprint Superoxide production is inversely related to complex I activity in inherited complex I deficiency
    Sjoerd Verkaart
    Department of Membrane Biochemistry, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Biochim Biophys Acta 1772:373-81. 2007
    ....