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

Stable Identifier
R-HSA-6783939
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
1-pyrroline-5-carboxylate + 2 H(+) + NADPH => L-proline + NADP(+)
ReviewStatus
5/5
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Pyrroline-5-carboxylate reductase 2 (PYCR2) catalyzes the reaction of (S)-1-pyrroline-5-carboxylate with NADPH + H+ to form proline and NADP+ (Merrill et al. 1989). The active enzyme is inferred to be a homodecamer by virtue of its similarity to PYCR1 (Meng et al. 2006). The enzyme is found in erythrocytes (Merrill et al. 1989), consistent with a cytosolic localization. The enzyme’s high affinity for NADPH and its inhibition by NADP+ but not by proline suggest that its physiological role is regulation of the NADP+/NADPH ratio and intracellular redox potential (Meng et al. 2006). 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
2722838 Purified human erythrocyte pyrroline-5-carboxylate reductase. Preferential oxidation of NADPH.

Phang, JM, Merrill, MJ, Yeh, GC

J Biol Chem 1989
Participants
Participates
Catalyst Activity

pyrroline-5-carboxylate reductase activity of PYCR2 decamer [cytosol]

Orthologous Events
Cross References
RHEA
Rhea
Authored
Reviewed
Created
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