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What exactly is the mechanism by which arginine supplementation can help with urea cycle disorders? And does it help with ALL causes of Urea cycle disorders d/t enzymatic deficiencies? (I believe there are 5 or so).
That does, I appreciate the added correlate of this being the mechanism by which arginine becomes essential. Is it not involved in the pathway of allowing for more urea production that would allow for increased NH3 excretion?
My question is basically- What is the mechanism by which we treat the hyperammonemia associated with urea cycle disorders?
So as far as I understand, there are no treatments to increase urea production. You treat it by minimizing ammonium production (limiting protein intake) and can also use benzoate, phenylacete, or phenylbuytrate which help clear glutamine really, however they do not increase urea production.
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Would you elaborate on that a little please- how would using Benzoate, phenylacetate, phenylbutyrate to increase Glutamine/Glycine clearance counteract the hyerammonemia? There's something I'm not getting.
My question is how exactly does the glutamate become the glutamine, that is worked on by the benzoate/phenylacetate/phenylbutyrate. I know theres a glutamate-glutamine cycle (astrocytes taking up excess glutamate from synaptic spaces and converting it to glutamine that they then pass back to neurons), but i dont think this is involved in this process, is it?
Again, really appreciate your attention to this.
From what ive learned, since glutamine basically has 2 NH3 groups (ie the things you want to get rid of in hyperammonemia), the phenylacetate just forms a complex with glutamine, forming phenylacetylglutamine which is excreted in the urine. (basically think of it as a chelating agent, even though its not sequestering metals)My question is how exactly does the glutamate become the glutamine, that is worked on by the benzoate/phenylacetate/phenylbutyrate. I know theres a glutamate-glutamine cycle (astrocytes taking up excess glutamate from synaptic spaces and converting it to glutamine that they then pass back to neurons), but i dont think this is involved in this process, is it?
Again, really appreciate your attention to this.