The PRODH gene provides instructions for producing the enzyme proline dehydrogenase (also known as proline oxidase), which is found primarily in the brain, lung, and muscle. Within cells of these organs, this enzyme functions in energy-producing structures called mitochondria.
Proline dehydrogenase plays a role in the process of breaking down the protein building block (amino acid) proline. Specifically, the enzyme starts the reaction that converts proline to pyrroline-5-carboxylate. A subsequent step converts this intermediate product to the amino acid glutamate.
The conversion of proline to glutamate (and the conversion of glutamate to proline, which is controlled by different enzymes) is important for maintaining a supply of amino acids needed for protein production, and for energy transfer within the cell.
Other Names
FLJ33744; HSPOX2; MGC148078; MGC148079; p53 induced protein; PIG6; PROD_HUMAN; PRODH1; PRODH2; proline dehydrogenase (oxidase) 1; proline dehydrogenase (proline oxidase ); proline oxidase 2; Proline oxidase, mitochondrial; SCZD4; TP53I6; tumor protein p53 inducible protein 6
Related Genetics Topics
Identifiers
- NCBI Gene: 5625
- OMIM: 606810
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
