Pyrroline-5-carboxylate reductase 1, mitochondrial

Definition:

Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress.

References:

[1]. Bruno Reversade, et al. Mutations in PYCR1 cause cutis laxa with progeroid features. Nat Genet. 2009 Sep;41(9):1016-21. [Content Brief]

[2]. Zhaohui Meng, et al. Crystal structure of human pyrroline-5-carboxylate reductase. J Mol Biol. 2006 Jun 23;359(5):1364-77. [Content Brief]

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