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

Stable Identifier
R-HSA-6783939
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
(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
9514741 Crystal structure of human recombinant ornithine aminotransferase.

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

J Mol Biol 1998
25865492 Mutations in PYCR2, Encoding Pyrroline-5-Carboxylate Reductase 2, Cause Microcephaly and Hypomyelination

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

Am. J. Hum. Genet. 2015
23024808 Functional specialization in proline biosynthesis of melanoma

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

PLoS ONE 2012
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
pyrroline-5-carboxylate reductase activity of PYCR2 decamer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created