Aliases for PRODH2 Gene
External Ids for PRODH2 Gene
Previous GeneCards Identifiers for PRODH2 Gene
The protein encoded by this gene catalyzes the first step in the catabolism of trans-4-hydroxy-L-proline, an amino acid derivative obtained through food intake and collagen turnover. One of the downstream products of this catabolism is glyoxylate, which in people with disorders of glyoxalate metabolism can lead to an increase in oxalate levels and the formation of calcium-oxalate kidney stones. Therefore, this gene may serve as a therapeutic target against primary hyperoxalurias (PH). This gene is similar to proline dehydrogenase (oxidase) 1, a mitochondrial enzyme that catalyzes the first step in proline catabolism. [provided by RefSeq, Jan 2017]
GeneCards Summary for PRODH2 Gene
PRODH2 (Proline Dehydrogenase 2) is a Protein Coding gene. Diseases associated with PRODH2 include Primary Hyperoxaluria and Maple Syrup Urine Disease, Type Ii. Among its related pathways are Arginine and proline metabolism and Metabolism. GO annotations related to this gene include proline dehydrogenase activity. An important paralog of this gene is LOC102724788.
UniProtKB/Swiss-Prot for PRODH2 Gene
Dehydrogenase that converts trans-4-L-hydroxyproline to delta-1-pyrroline-3-hydroxy-5-carboxylate (Hyp) using ubiquinone-10 as the terminal electron acceptor. Can also use proline as a substrate but with a very much lower efficiency. Does not react with other diastereomers of Hyp: trans-4-D-hydroxyproline and cis-4-L-hydroxyproline. Ubiquininone analogs such as menadione, duroquinone and ubiquinone-1 react more efficiently than oxygen as the terminal electron acceptor during catalysis.