1-pyrroline-5-carboxylate dehydrogenase

1-pyrroline-5-carboxylate dehydrogenase
An enzyme that catalyzes the reversible reaction of 1-pyrroline 5-carboxylate and NAD+ to form l-glutamate and NADH; this enzyme plays a role in proline and ornithine metabolism; 1-pyrroline 5-carboxylate is in equilibrium with glutamate γ-semialdehyde; a deficiency of this enzyme is associated with type II hyperprolinemia.

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1-pyr·ro·line-5-car·box·y·late de·hy·dro·gen·ase (pirґo-lēn kahr-bokґsə-lāt de-hiґdro-jən-ās) [EC 1.5.1.12] an enzyme of the oxidoreductase class that catalyzes the dehydrogenation of Δ1-pyrroline 5-carboxylate to glutamate, using NAD+ as an electron acceptor; the reaction is a step in the degradation of proline and excess ornithine. The enzyme can also oxidize the 3-hydroxy derivative of Δ1-pyrroline 5-carboxylate to form 4-hydroxyglutamate. Deficiency of the enzyme, an autosomal recessive trait, is the cause of hyperprolinemia, type II.

Medical dictionary. 2011.

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