pyrroline-5-carboxylate reductase 1, mitochondrial; P5C reductase 1; P5CR 1 (PYCR1)

Function: - catalyzes the terminal step in the biosynthesis of proline from glutamate, the NAD(P) dependent oxidation of 1-pyrroline-5-carboxylate into proline - role in the cellular response to oxidative stress - amino-acid biosynthesis; L-proline biosynthesis; L-proline from L-glutamate 5-semialdehyde: step 1/1 - homodecamer; composed of 5 homodimers L-proline + NAD+/NADP+ = 1-pyrroline-5-carboxylate + NADH/NADPH Structure: - belongs to the pyrroline-5-carboxylate reductase family Compartment: mitochondria Pathology: - defects in PYCR1 are the cause of cutis laxa type-2B & De Barsy syndrome

Properties

SIZE: entity length = 319 aa MW = 33 kD COMPARTMENT: mitochondria MOTIF: acetylation site SITE: N-TERMINUS EFFECTOR-BOUND: acetyl

Database Correlations

OMIM correlations MORBIDMAP 179035 UniProt P32322 Pfam PF03807 Entrez Gene 5831 Kegg hsa:5831 ENZYME 1.5.1.2

References

UniProt :accession P32322