dbId
178924
displayName
Pyrroline-5-carboxylate reductase 1 (PYCR1) catalyzes the re...
schemaClass
Summation
text
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).