Glutamate dehydrogenase
Clinical / pharmacological. Activating mutations: hyperinsulinism-hyperammonaemia syndrome
What it does, reaction by reaction
Oxidative Deamination Amino Acid & Nitrogen Metabolism · Mitochondrial matrix
L-glutamate + NAD⁺ (or NADP⁺) + H₂O ⇌ α-ketoglutarate + NH₄⁺ + NADH (or NADPH) + H⁺
Converts Glutamate NAD+ (or NADP+) H2O into Alpha-ketoglutarate NH4+ NADH (or NADPH) H+
› Notes
Glutamate dehydrogenase catalyzes oxidative deamination in the mitochondrial matrix. NAD⁺ is generally the major oxidant during net hepatic glutamate oxidation, although the enzyme can use NADP⁺; the reaction is reversible and is the principal regulated route for release of free ammonia from glutamate.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
Oxidative Deamination
ADP, GDP; leucine allosterically activates the mammalian enzyme
ATP, GTP; high-energy charge
Glucagon and cortisol increase hepatic amino-acid catabolic/ureagenic capacity indirectly; insulin suppresses net amino-acid catabolism in the fed state
Recent literature
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