pyrroline-5-carboxylate reductase 1, mitochondrial; P5C reductase 1; P5CR 1 (PYCR1)
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Function
- catalyzes the terminal step in the biosynthesis of proline from glutamate, the NAD(P) dependent oxidation of 1-pyrroline-5-carboxylate into proline
- role in the cellular response to oxidative stress
- amino-acid biosynthesis; L-proline biosynthesis; L-proline from L-glutamate 5-semialdehyde: step 1/1
- homodecamer; composed of 5 homodimers
L-proline + NAD+/NADP+ = 1-pyrroline-5-carboxylate + NADH/NADPH
Structure
- belongs to the pyrroline-5-carboxylate reductase family
Compartment
Pathology
- defects in PYCR1 are the cause of cutis laxa type-2B & De Barsy syndrome