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Reviewed July 2007
What is the official name of the PCCB gene?
The official name of this gene is “propionyl-CoA carboxylase beta subunit.”
PCCB is the gene's official symbol. The PCCB 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 PCCB gene?
The PCCB gene provides instructions for making part of an enzyme called propionyl-CoA carboxylase, specifically, the beta subunit of this enzyme. Six beta subunits come together with six alpha subunits (produced from the PCCA gene) to form a functioning enzyme.
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 the B vitamin 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 PCCB gene related to health conditions?
Where is the PCCB gene located?
Cytogenetic Location: 3q21-q22
Molecular Location on chromosome 3: base pairs 136,250,325 to 136,330,171
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The PCCB gene is located on the long (q) arm of chromosome 3 between positions 21 and 22.
More precisely, the PCCB gene is located from base pair 136,250,325 to base pair 136,330,171 on chromosome 3.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about PCCB?
You and your healthcare professional may find the following resources about PCCB 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 PCCB 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 PCCB?
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (5 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.