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Browsing by Subject N-acetylglutamate synthase

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  • OER000000647.pdf.jpg
  • Journal article

  • Mitochondrial Enzymes of the Urea Cycle Cluster at the Inner Mitochondrial Membrane

    • Authors : Haskins, Nantaporn; Bhuvanendran, Shivaprasad; Gams, Anna (2020)

  • Mitochondrial enzymes involved in energy transformation are organized into multiprotein complexes that channel the reaction intermediates for efficient ATP production. Three of the mammalian urea cycle enzymes: N-acetylglutamate synthase (NAGS), carbamylphosphate synthetase 1 (CPS1), and ornithine transcarbamylase (OTC) reside in the mitochondria. Urea cycle is required to convert ammonia into urea and protect the brain from ammonia toxicity. Urea cycle intermediates are tightly...