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A Liver-Specific Defect of Acyl-CoA Degradation Produces Hyperammonemia, Hypoglycemia and a Distinct Hepatic Acyl-CoA Pattern

  • Nicolas Gauthier,

    Affiliations Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada, Département de Biochimie, Université de Montréal, Montréal, Québec, Canada

  • Jiang Wei Wu,

    Affiliation Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

  • Shu Pei Wang,

    Affiliation Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

  • Pierre Allard,

    Affiliation Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

  • Orval A. Mamer,

    Affiliation Goodman Cancer Research Centre, McGill University, Montréal, Québec, Canada

  • Lawrence Sweetman,

    Affiliation Institute of Metabolic Disease, Baylor Research Institute, Dallas, Texas, United States of America

  • Ann B. Moser,

    Affiliation The Hugo W Moser Research Institute, The Kennedy-Krieger Institute, Johns Hopkins School of Medicine, Baltimore, Maryland, United States of America

  • Lisa Kratz,

    Affiliation The Hugo W Moser Research Institute, The Kennedy-Krieger Institute, Johns Hopkins School of Medicine, Baltimore, Maryland, United States of America

  • Fernando Alvarez,

    Affiliation Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

  • Yves Robitaille,

    Affiliation Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada

  • François Lépine,

    Affiliation INRS-Institut Armand-Frappier, Laval, Québec, Canada

  • Grant A. Mitchell

    grant.mitchell@recherche-ste-justine.qc.ca

    Affiliations Centre de Recherche and Département de Pédiatrie (NG, JWW, SPW, PA, FA, GAM) or Département de Pathologie (YR), CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada, Département de Biochimie, Université de Montréal, Montréal, Québec, Canada

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