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Reviewed July 2007
What is the official name of the PCCA gene?
The official name of this gene is “propionyl-CoA carboxylase alpha subunit.”
PCCA is the gene's official symbol. The PCCA 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 PCCA gene?
The PCCA gene provides instructions for making part of an enzyme called propionyl-CoA carboxylase, specifically, the alpha subunit of this enzyme. Six alpha subunits come together with six beta subunits (produced from the PCCB gene) to form a functioning enzyme. The alpha subunit also includes a region for binding to the B vitamin biotin.
Propionyl-CoA carboxylase plays a role in the normal processing of proteins. It is responsible for a particular step in the breakdown of several protein building blocks (amino acids) called isoleucine, methionine, threonine, and valine. Propionyl-CoA carboxylase also helps break down certain types of lipids (fats) and cholesterol. First, several chemical reactions convert the amino acids, lipids, or cholesterol to a molecule called propionyl-CoA. Using biotin, propionyl-CoA carboxylase then converts propionyl-CoA to a molecule called methylmalonyl-CoA. Additional enzymes break down methylmalonyl-CoA into other molecules that are used for energy.
How are changes in the PCCA gene related to health conditions?
Where is the PCCA gene located?
Cytogenetic Location: 13q32
Molecular Location on chromosome 13: base pairs 100,089,015 to 100,530,437
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The PCCA gene is located on the long (q) arm of chromosome 13 at position 32.
More precisely, the PCCA gene is located from base pair 100,089,015 to base pair 100,530,437 on chromosome 13.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about PCCA?
You and your healthcare professional may find the following resources about PCCA 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 PCCA 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 PCCA?
acids ; biotin ; breakdown ; carboxylase ; cholesterol ; CoA ; coenzyme A ; DNA ; enzyme ; gene ; isoleucine ; ligase ; methionine ; molecule ; nervous system ; protein ; subunit ; threonine ; toxic ; valine
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (5 links)