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Comprehensive characterization of ureagenesis in the spfash mouse, a model of human ornithine transcarbamylase deficiency, reveals age-dependency of ammonia detoxification.
Allegri, Gabriella; Deplazes, Sereina; Rimann, Nicole; Causton, Benjamin; Scherer, Tanja; Leff, Jonathan W; Diez-Fernandez, Carmen; Klimovskaia, Anna; Fingerhut, Ralph; Krijt, Jakub; Kozich, Viktor; Nuoffer, Jean-Marc; Grisch-Chan, Hiu M; Thöny, Beat; Häberle, Johannes.
Afiliación
  • Allegri G; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Deplazes S; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Rimann N; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Causton B; Kaleido Biosciences Inc., Lexington, Massachusetts.
  • Scherer T; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Leff JW; Kaleido Biosciences Inc., Lexington, Massachusetts.
  • Diez-Fernandez C; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Klimovskaia A; Institute for Molecular Systems Biology, ETH Zurich, Zurich, Switzerland.
  • Fingerhut R; Swiss Newborn Screening Laboratory, University Children's Hospital, Zurich, Switzerland.
  • Krijt J; Department of Pediatrics and Adolescent Medicine, Charles University-First Faculty of Medicine and General University Hospital in Prague, Prague, Czech Republic.
  • Kozich V; Department of Pediatrics and Adolescent Medicine, Charles University-First Faculty of Medicine and General University Hospital in Prague, Prague, Czech Republic.
  • Nuoffer JM; Department of Clinical Chemistry, Inselspital Bern, Bern, Switzerland.
  • Grisch-Chan HM; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Thöny B; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
  • Häberle J; Division of Metabolism and Children's Research Center (CRC), University Children's Hospital Zurich, Zurich, Switzerland.
J Inherit Metab Dis ; 42(6): 1064-1076, 2019 11.
Article en En | MEDLINE | ID: mdl-30714172

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Ornitina Carbamoiltransferasa / Urea / Envejecimiento / Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa / Amoníaco Tipo de estudio: Prognostic_studies Límite: Animals / Humans / Male Idioma: En Año: 2019 Tipo del documento: Article

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Ornitina Carbamoiltransferasa / Urea / Envejecimiento / Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa / Amoníaco Tipo de estudio: Prognostic_studies Límite: Animals / Humans / Male Idioma: En Año: 2019 Tipo del documento: Article