PYCR1 decamer reduces (S)-1-pyrroline-5-carboxylate to L-Pro

Stable Identifier
R-HSA-70664
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
(S)-1-pyrroline-5-carboxylate + NADH + H+ => L-Pro + NAD+
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Pyrroline-5-carboxylate reductase 1 (PYCR1) 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 a homodecamer (Meng et al. 2006). Subcellular fractionation (De Ingeniis et al. 2012) and co-localization studies (Reversade et al. 2009) indicate that PYCR1 is mitochondrial. Its deficiency is associated with cutis laxa (Reversade et al. 2009).

Literature References
PubMed ID Title Journal Year
19648921 Mutations in PYCR1 cause cutis laxa with progeroid features

Seemann, P, Schmidt-von Kegler, M, Reversade, B, Hausser, I, Masri, A, Tham, PY, Li, Y, Schuelke, M, Kayserili, H, Brancati, F, Nürnberg, G, Wollnik, B, Sillence, D, Zambruno, G, Nelson, SF, Chng, SC, Mundlos, S, Nanda, A, Grix, A, Alkazaleh, F, Savarirayan, R, Guerra, D, Escande-Beillard, N, Kunkel, D, Shboul, M, Lee, H, Dimopoulou, A, Markie, D, Ferrari, P, Budde, B, Shahwan, M, Hamamy, H, Nelson, J, Gray, M, Steichen, E, Dallapiccola, B, Kornak, U, Merriman, B, Van Maldergem, L, Robertson, S, Al-Gazali, L, Rajab, A, Fischer, B, Sommer, A, O'Connor, BD, Janecke, AR, Nürnberg, P

Nat Genet 2009
16730026 Crystal structure of human pyrroline-5-carboxylate reductase

Bartlam, M, Li, M, Rao, Z, Lou, Z, Zhao, X, Meng, Z, Liu, Z

J Mol Biol 2006
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
Participants
Participates
Catalyst Activity

pyrroline-5-carboxylate reductase activity of PYCR1 decamer [mitochondrial matrix]

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