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

Stable Identifier
R-HSA-70664
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
(S)-1-pyrroline-5-carboxylate + NADH + H+ => L-Pro + NAD+
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
16730026 Crystal structure of human pyrroline-5-carboxylate reductase

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

J Mol Biol 2006
19648921 Mutations in PYCR1 cause cutis laxa with progeroid features

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

Nat Genet 2009
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 PYCR1 decamer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events