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

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

Stable Identifier

R-HSA-70664

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

1-pyrroline-5-carboxylate + 2 H(+) + NADH => L-proline + NAD(+)

ReviewStatus

5/5

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

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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. The enzyme’s higher reaction rate with NADH than NADPH as cofactor, and its competitive inhibition by the product proline suggest that its main physiological function is production of proline (Meng et al. 2006). 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