PYCR2 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro

Stable Identifier
Reaction [transition]
Homo sapiens
(S)-1-pyrroline-5-carboxylate + NADH + H+ => L-Pro + NAD+
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Pyrroline-5-carboxylate reductase 2 (PYCR2) catalyzes the reaction of (S)-1-pyrroline-5-carboxylate with NADH + H+ to form proline and NAD+ (De Ingeniis et al. 2012). The active enzyme is inferred to be a homodecamer by virtue of its similarity to PYCR1 (Meng et al. 2006). Subcellular fractionation (De Ingeniis et al. 2012) and co-localization studies (Nakayama et al. 2015) indicate that PYCR1 is mitochondrial. Its deficiency is associated with microcephaly and hypomyelination (Nakayama et al. 2015).
Literature References
PubMed ID Title Journal Year
25865492 Mutations in PYCR2, Encoding Pyrroline-5-Carboxylate Reductase 2, Cause Microcephaly and Hypomyelination

Gorski, G, Poduri, A, Tambunan, DE, El-Quessny, M, Elhosary, PC, Servattalab, S, Mochida, GH, Barkovich, AJ, Al-Saffar, M, Markianos, K, LaCoursiere, CM, Coulter, ME, Schmitz-Abe, K, Tan, WH, Khalil, S, Rajab, A, Nakayama, T, Al-Maawali, A, Stoler, JM, Hill, RS, Partlow, JN, Nasir, R

Am. J. Hum. Genet. 2015
9514741 Crystal structure of human recombinant ornithine aminotransferase.

Schirmer, T, Hohenester, E, Jansonius, JN, Shen, BW, Hennig, M

J Mol Biol 1998
23024808 Functional specialization in proline biosynthesis of melanoma

Aza-Blanc, P, Scott, DA, Smith, JW, Osterman, AL, De, SK, Ratnikov, B, Ronai, Z, De Ingeniis, J, Richardson, AD, Kazanov, M, Pellecchia, M

PLoS ONE 2012
Catalyst Activity

pyrroline-5-carboxylate reductase activity of PYCR2 decamer [mitochondrial matrix]

Orthologous Events
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