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A Constitutive Knockout of Murine Carbamoyl Phosphate Synthetase 1 Results in Death with Marked Hyperglutaminemia and Hyperammonemia

The enzyme carbamoyl phosphate synthetase 1 (CPS1; EC 6.3.4.16) forms carbamoyl phosphate from bicarbonate, ammonia, and ATP and is activated allosterically by N-acetylglutamate. The neonatal presentation of bi-allelic mutations of CPS1 results in hyperammonemia with reduced citrulline and is report...

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Detalhes bibliográficos
Publicado no:J Inherit Metab Dis
Main Authors: Khoja, Suhail, Nitzahn, Matthew, Truong, Brian, Lambert, Jenna, Willis, Brandon, Allegri, Gabriella, Rüfenacht, Véronique, Häberle, Johannes, Lipshutz, Gerald S.
Formato: Artigo
Idioma:Inglês
Publicado em: 2019
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6728231/
https://ncbi.nlm.nih.gov/pubmed/30835861
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jimd.12048
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