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

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(S)-1-pyrroline-5-carboxylate + NADH + H+ => L-Pro + NAD+

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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 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