Proline oxidase

Proline dehydrogenase, mitochondrial is an is_associated_with::enzyme that in humans is encoded by the PRODH is_associated_with::gene.

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