Proline Dehydrogenase, Mitochondrial (PRODH)

Proline dehydrogenase, mitochondrial is a mitochondrial proline dehydrogenase which catalyzes the first step in proline catabolism. Deletion of this gene has been associated with type I hyperprolinemia. The gene is located on chromosome 22q11.21, a region which has also been associated with the contiguous gene deletion syndromes: DiGeorge syndrome and CATCH22 syndrome.

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-NH group of donors with other acceptors.This enzyme participates in arginine and proline metabolism. the two substrates of this enzyme are L-proline and acceptor, whereas its two products are (S)-1-pyrroline-5-carboxylate and reduced acceptor.