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Research Article

Mitochondrial 2,4-dienoyl-CoA Reductase Deficiency in Mice Results in Severe Hypoglycemia with Stress Intolerance and Unimpaired Ketogenesis

  • Ilkka J. Miinalainen,

    Affiliation: Department of Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland

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  • Werner Schmitz,

    Affiliation: Theodor-Boveri-Institut für Biowissenschaften (Biozentrum) der Universität Würzburg, Würzburg, Germany

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  • Anne Huotari,

    Affiliation: Department of Biotechnology and Molecular Medicine, A.I. Virtanen Institute for Molecular Sciences, Kuopio, Finland

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  • Kaija J. Autio,

    Affiliation: Department of Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland

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  • Raija Soininen,

    Affiliation: Department of Medical Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland

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  • Emiel Ver Loren van Themaat,

    Affiliation: Bioinformatics Laboratory, Department of Clinical Epidemiology, Biostatistics and Bioinformatics, Academic Medical Center, Amsterdam, The Netherlands

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  • Myriam Baes,

    Affiliation: Laboratory of Cell Metabolism, Department of Pharmaceutical Sciences, Katholieke Universiteit Leuven, Leuven, Belgium

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  • Karl-Heinz Herzig,

    Affiliations: Department of Biotechnology and Molecular Medicine, A.I. Virtanen Institute for Molecular Sciences, Kuopio, Finland, Department of Internal Medicine, Kuopio and Institute of Biomedicine, Division of Physiology and Biocenter of Oulu, Oulu University Medical School, Oulu, Finland

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  • Ernst Conzelmann,

    Affiliation: Theodor-Boveri-Institut für Biowissenschaften (Biozentrum) der Universität Würzburg, Würzburg, Germany

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  • J. Kalervo Hiltunen mail

    Kalervo.Hiltunen@oulu.fi

    Affiliation: Department of Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland

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  • Published: July 03, 2009
  • DOI: 10.1371/journal.pgen.1000543

About the Authors

Ilkka J. Miinalainen, Kaija J. Autio, J. Kalervo Hiltunen
Department of Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland
Werner Schmitz, Ernst Conzelmann
Theodor-Boveri-Institut für Biowissenschaften (Biozentrum) der Universität Würzburg, Würzburg, Germany
Anne Huotari, Karl-Heinz Herzig
Department of Biotechnology and Molecular Medicine, A.I. Virtanen Institute for Molecular Sciences, Kuopio, Finland
Raija Soininen
Department of Medical Biochemistry and Biocenter Oulu, University of Oulu, Oulu, Finland
Emiel Ver Loren van Themaat
Bioinformatics Laboratory, Department of Clinical Epidemiology, Biostatistics and Bioinformatics, Academic Medical Center, Amsterdam, The Netherlands
Myriam Baes
Laboratory of Cell Metabolism, Department of Pharmaceutical Sciences, Katholieke Universiteit Leuven, Leuven, Belgium
Karl-Heinz Herzig
Department of Internal Medicine, Kuopio and Institute of Biomedicine, Division of Physiology and Biocenter of Oulu, Oulu University Medical School, Oulu, Finland

Corresponding Author

Email: Kalervo.Hiltunen@oulu.fi

Competing Interests

The authors have declared that no competing interests exist.

Author Contributions

Conceived and designed the experiments: IJM WS AH KJA RS EVLvT MB KHH EC JKH. Performed the experiments: IJM WS AH KJA EVLvT KHH. Analyzed the data: IJM WS AH KJA RS EVLvT MB KHH EC JKH. Contributed reagents/materials/analysis tools: RS MB JKH. Wrote the paper: IJM WS AH RS EVLvT KHH EC JKH.