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

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

Stable Identifier

R-HSA-6783939

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

1-pyrroline-5-carboxylate + 2 H(+) + NADPH => L-proline + NADP(+)

ReviewStatus

5/5

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

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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