Figure 1.
Metabolic Pathways for proline and hydroxyproline. Proline and hydroxyproline are metabolized by distinct dehydrogenases, the products are pyrroline-5-carboxylate and OH-pyrroline-5-carboxylate, respectively. Proline dehydrogenase (PRODH) and Hydroxyproline dehydrogenase (PRODH2) are distinct gene products but both are bound to mitochondrial inner membranes and donate electrons to FAD at site II. PCDH is in mitochondrial matrix and converts P5C and OH-P5C to glutamate and OH-glutamate, respectively. Both P5C and OH-P5C can be recycled to proline and hydroxyproline, respectively by generic PYCR. Since 3 isozymes of PYCR are known, preferential affinity of the isozymes for P5C and OH-P5C has not been defined. Competition between P5C and OH-P5C for PYCR1-3 and for PCDH has not been examined.