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Reviewed July 2009
What is the official name of the UROD gene?
The official name of this gene is “uroporphyrinogen decarboxylase.”
UROD is the gene's official symbol. The UROD 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 UROD gene?
The UROD gene provides instructions for making an enzyme known as uroporphyrinogen decarboxylase. This enzyme is involved in the production of a molecule called heme. Heme is vital for all of the body's organs, although it is most abundant in the blood, bone marrow, and liver. Heme is an essential component of iron-containing proteins called hemoproteins, including hemoglobin (the protein that carries oxygen in the blood).
The production of heme is a multi-step process that requires eight different enzymes. Uroporphyrinogen decarboxylase is responsible for the fifth step in this process, in which carbon and oxygen atoms are removed from uroporphyrinogen III (the product of the fourth step) to form coproporphyrinogen III. In subsequent steps, three other enzymes produce and modify compounds that ultimately lead to heme.
How are changes in the UROD gene related to health conditions?
Where is the UROD gene located?
Cytogenetic Location: 1p34
Molecular Location on chromosome 1: base pairs 45,012,133 to 45,015,669
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The UROD gene is located on the short (p) arm of chromosome 1 at position 34.
More precisely, the UROD gene is located from base pair 45,012,133 to base pair 45,015,669 on chromosome 1.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about UROD?
You and your healthcare professional may find the following resources about UROD 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 UROD 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 UROD?
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (8 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.