Recombinant Human Probable proline dehydrogenase 2 (PRODH2)

Uniprot Id

Q9UF12

Target Species

Human

Target Name

PRODH2

Target Full Name

Hydroxyproline dehydrogenase

Target Function

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.

Target Protein Families

Proline oxidase family

Target Synonyms

HsPOX1; Kidney and liver proline oxidase 1; Probable proline dehydrogenase 2; Probable proline oxidase 2; PROD2_HUMAN; PRODH2

Target Background

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.