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Reviewed June 2007
What is the official name of the PRODH gene?
The official name of this gene is “proline dehydrogenase (oxidase) 1.”
PRODH is the gene's official symbol. The PRODH gene is also known by other names, listed below.
Read more about gene names and symbols on the About page.
What is the normal function of the PRODH gene?
The PRODH gene provides instructions for producing the enzyme proline oxidase, which is found primarily in the brain, liver, and kidney. Within cells of these organs, this enzyme functions in energy-producing structures called mitochondria.
Proline oxidase begins the process of breaking down the protein building block (amino acid) proline by starting the reaction that converts it to pyrroline-5-carboxylate. A subsequent step converts this intermediate product to the amino acid glutamate. The conversion between proline and glutamate is important in maintaining a supply of the amino acids needed for protein production, and for energy transfer within the cell.
How are changes in the PRODH gene related to health conditions?
Where is the PRODH gene located?
Cytogenetic Location: 22q11.21
Molecular Location on chromosome 22: base pairs 18,912,695 to 18,936,553
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The PRODH gene is located on the long (q) arm of chromosome 22 at position 11.21.
More precisely, the PRODH gene is located from base pair 18,912,695 to base pair 18,936,553 on chromosome 22.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about PRODH?
You and your healthcare professional may find the following resources about PRODH helpful.
You may also be interested in these resources, which are designed for genetics professionals and researchers.
What other names do people use for the PRODH gene or gene products?
See How are genetic conditions and genes named? in the Handbook.
Where can I find general information about genes?
The Handbook provides basic information about genetics in clear language.
These links provide additional genetics resources that may be useful.
What glossary definitions help with understanding PRODH?
acids ; amino acid ; breakdown ; carboxylate ; cell ; degradation ; dehydrogenase ; disability ; enzyme ; gene ; kidney ; mitochondria ; neurological ; neurotransmitters ; oxidase ; proline ; protein ; schizophrenia ; substitution ; tumor
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (14 links)
The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? in the Handbook.