PRODH (proline dehydrogenase), also known as proline oxidase 2 (POX2), is a 516 amino acid protein belonging to the proline oxidase family. Induced during p53-induced apoptosis, PRODH catalyzes the first step in proline degradation by converting proline to delta-1-pyrroline-5-carboxylate. Defects in PRODH are the cause of hyperprolinemia type 1, a disorder characterized by elevated serum proline levels. Defects in PRODH may also be involved in the psychiatric and behavioral phenotypes associated with DiGeorge syndrome. Localized to the mitochondrion matrix, PRODH is expressed at higher levels in lung, brain and skeletal muscle and expressed at lower ...
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