1 to 7 of 7 literature results

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

  Ecsit is a cytosolic adaptor protein essential for inflammatory response and embryonic development via the Toll-like and BMP (bone morphogenetic prote…

  Authors: Rutger O Vogel, Rolf J R J Janssen, Mariël A M van den Brand, Cindy E J Dieteren, Sjoerd Verkaart, Werner J H Koopman, Peter H G M Willems, Wendy Pluk, Lambert P W J van den Heuvel, Jan A M Smeitink,…

  Source: Genes & development. 2007 Mar 1

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• NDUFA2 complex I mutation leads to Leigh disease.

  Mitochondrial isolated complex I deficiency is the most frequently encountered OXPHOS defect. We report a patient with an isolated complex I deficienc…

  Authors: Saskia J G Hoefs, Cindy E J Dieteren, Felix Distelmaier, Rolf J R J Janssen, Andrea Epplen, Herman G P Swarts, Marleen Forkink, Richard J Rodenburg, Leo G Nijtmans, Peter H Willems,…

  Source: American journal of human genetics. 2008 Jun

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• Baculovirus complementation restores a novel NDUFAF2 mutation causing complex I deficiency.

  Mitochondrial complex I deficiency is the most common defect of the OXPHOS system. We report a patient from consanguineous parents with a complex I de…

  Authors: Saskia J G Hoefs, Cindy E J Dieteren, Richard J Rodenburg, Karin Naess, Helene Bruhn, Rolf Wibom, Esther Wagena, Peter H Willems, Jan A M Smeitink, Leo G Nijtmans,…

  Source: Human mutation. 2009 Jul

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• Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation.

  Virtually every mammalian cell contains mitochondria. These double-membrane organelles continuously change shape and position and contain the complete…

  Authors: Werner J H Koopman, Leo G J Nijtmans, Cindy E J Dieteren, Peggy Roestenberg, Federica Valsecchi, Jan A M Smeitink, Peter H G M Willems

  Source: Antioxidants & redox signaling. 2010 Jun 15

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• Chapter 7 Tracing human mitochondrial complex I assembly by use of GFP-tagged subunits.

  Disturbances in the assembly of mitochondrial complex I (CI) are a frequent cause of mitochondrial disorders. Several lines of evidence hint at a semi…

  Authors: Cindy E J Dieteren, Werner J H Koopman, Leo G J Nijtmans

  Source: Methods in enzymology. 2009

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• Subunits of mitochondrial complex I exist as part of matrix- and membrane-associated subcomplexes in living cells.

  Mitochondrial complex I (CI) is a large assembly of 45 different subunits, and defects in its biogenesis are the most frequent cause of mitochondrial …

  Authors: Cindy E J Dieteren, Peter H G M Willems, Rutger O Vogel, Herman G Swarts, Jack Fransen, Ronald Roepman, Gijs Crienen, Jan A M Smeitink, Leo G J Nijtmans, Werner J H Koopman

  Source: The Journal of biological chemistry. 2008 Dec 12

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

  Biogenesis of human mitochondrial complex I (CI) requires the coordinated assembly of 45 subunits derived from both the mitochondrial and nuclear geno…

  Authors: Rutger O Vogel, Cindy E J Dieteren, Lambert P W J van den Heuvel, Peter H G M Willems, Jan A M Smeitink, Werner J H Koopman, Leo G J Nijtmans

  Source: The Journal of biological chemistry. 2007 Mar 9

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