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l-Proline metabolism and enzyme deficiencies. The metabolism of proline in mammals involves two other amino acids, glutamate and ornithine, and six enzymes. Conversion of P5C to glutamic-γ-semi-aldehyde is a non-enzymatic step. HPI, hyperprolinemia type I; HPII, hyperprolinemia type II; P5C, Δ-1-pyrroline-5-carboxylate; TCA, tricarboxylic acid.
