| pyrroline 5-carboxylate synthase |
a mitochondrial enzyme activity that catalyzes the conversion of glutamate to Δ1-pyrroline 5-carboxylate, an intermediate in the synthesis of proline. ATP is hydrolyzed in the reaction and NADPH is the electron donor.
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| pyrroline-5-carboxylate reductase |
[EC 1.5.1.2] an enzyme of the oxidoreductase class that catalyzes the irreversible reduction of Δ1-pyrroline 5-carboxylate to proline, using NADPH (or NADH) as an electron donor; the reaction is the final step in the biosynthesis of proline from glutamate or ornithine. The enzyme may also reduce the 3-hydroxy analog of pyrroline 5-carboxylate to form hydroxyproline.
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| pyrroloporphyria |
acute intermittent porphyria.
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