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• 14:15, 10 January 2023 Nikolas talk contribs created page 15. Enzymopathies of amino acid metabolism (Created page with "== Learning objectives == * Why is ammonia toxic to the brain? * Which enzyme is defect in the diseases mentioned? == Hyperammonaemia == 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 in two ways. The first is by the ''glutamate dehydrogenase'' reaction, which converts α-ketoglutarate and ammonium to glutamate. However, thi...") Tag: Visual edit