15. Enzymopathies of amino acid metabolism

Last updated on November 19, 2018 at 17:16


  • I’ve outlined the diseases you should know in a separate topic
  • Hyperammonemia is a result of urea cycle defects, or permanent activation of glutamate dehydrogenase

Ammonia is bad

As outlined before, too much ammonia is very bad. It can easily cross the blood-brain barrier, so it is toxic to the brain. If the brain contains too much ammonia, it will try to get rid of it by converting glutamate to glutamine, which consumes ammonia. However, this will deplete storage of glutamate, which is the direct precursor to GABA, an important neurotransmitter.

Hyperammonemia is a result of either urea cycle defects, as the body has trouble excreting ammonia, or by permanent activation of glutamate dehydrogenase, which breaks down glutamate into α-ketoglutarate and ammonia.

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14. Synthesis of non-essential amino acids

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16. Synthesis of biologically active molecules from amino acids

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