proline oxidase (proline dehydrogenase, PRODH)

Function

• converts proline to delta-1-pyrroline-5-carboxylate
• amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 1/2

L-proline + acceptor + H2O
           <-->
(S)-1-pyrroline-5-carboxylate + reduced acceptor

Cofactor: FAD

Structure

belongs to the proline oxidase family

Compartment

mitochondria matrix

Alternative splicing

named isoforms=5

Expression

• expressed in lung, skeletal muscle & brain
• lesser amounts in heart & kidney, & weakly in liver, placenta & pancreas
• expressed during TP53-induced apoptosis

Pathology

• defects in PRODH are the cause of hyperprolinemia type 1
• defects in PRODH may be associated with susceptibility to schizophrenia 4

Polymorphism

• variant in position: 8:P->L
• variant in position: 19:Q->P (moderate reduction of enzymatic activity, dbSNP:rs2008720)
• variant in position: 30:P->S
• variant in position: 58:A->T

More general terms

• amine oxidoreductase
• dehydrogenase

Database

• UniProt: http://www.uniprot.org/uniprot/O43272.html
• OMIM: https://mirror.omim.org/entry/181500
• OMIM: https://mirror.omim.org/entry/239500
• OMIM: https://mirror.omim.org/entry/600850
• OMIM: https://mirror.omim.org/entry/606810