Proline Dehydrogenase 2 (PRODH2)

PRODH2 is similar to proline dehydrogenase (oxidase) 1, a mitochondrial enzyme which catalyzes the first step in proline catabolism. PRODH2, is the likely cause of schizophrenia susceptibility. PRODH2 encodes proline dehydrogenase, a mitochondrial enzyme that converts proline to Δ¹-pyrroline-5-carboxylate and is involved in transfer of redox potential across the mitochondrial membrane. The gene is widely expressed in many tissues, including in the brain, and its homozygous deficiency, hyperprolinemia, is an inborn error of metabolism. There is some indication that heterozygous deficiency of PRODH2 may be a cause of isolated hyperprolinemia. Importantly, mice created to be homozygous for PRODH2 deficiency demonstrate a deficit in prepulse inhibition, a measure of sensorimotor gating affected in schizophrenics.