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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
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
Pathology:
- defects in PRODH are the cause of hyperprolinemia type 1
- defects in PRODH may be associated with susceptibility to schizophrenia 4
General
amine oxidoreductase
dehydrogenase
Properties
SIZE: entity length = 516 aa
MW = 59 kD
COMPARTMENT: mitochondria
MOTIF: active site
Database Correlations
OMIM correlations
MORBIDMAP 606810
UniProt O43272
Pfam PF01619
ENZYME 1.5.99.8
References
- UniProt :accession O43272
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=PRODH