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

More general terms

• oxidoreductase

Additional terms

• pyrroline-5-carboxylate reductase 2; P5CR 2; P5C reductase 2 (PYCR2)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=5831
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5831
• OMIM: https://mirror.omim.org/entry/179035
• OMIM: https://mirror.omim.org/entry/612940
• UniProt: http://www.uniprot.org/uniprot/P32322.html