Table 6.

Mechanisms of Drug Action in Treatment of Hyperammonemia

DrugAction
Glucose
  • Raises insulin levels
  • Induces anabolic state
  • Causes protein sparing effect from skeletal muscle amino acids
Arginine 1
  • Needs to be supplemented in those w/a urea cycle disorder
  • Stimulates secretion of insulin
  • Plays a role in 1st step of creatine synthesis 2
Sodium benzoate 3
  • Forms benzoate-glycine (hippurate) via the benzoylCoA:glycine acyltransferase reaction 4
  • Eliminates 1 amino group in the urine
Sodium phenylacetate 3
  • Forms a phenylacetate-glutamine compound via the phenylacetateCoA:glutamine acetyl-transferase reaction 4
  • Eliminates 2 amino groups in the urine
1.

A nonessential amino acid in humans

2.

Interruption in the synthesis of brain creatine secondary to hyperammonemia has been proposed as a contributing factor to the neurologic findings in affected individuals.

3.

Initially esterified to its CoA-ester via the medium-chain fatty acid enzyme acyl-CoA ligase

4.

Reaction takes place in the mitochondrial matrix (liver and kidney) [Brusilow & Horwich 2001].

From: Hyperornithinemia-Hyperammonemia-Homocitrullinuria Syndrome

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