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Reviewed December 2008
What is the official name of the AGA gene?
The official name of this gene is “aspartylglucosaminidase.”
AGA is the gene's official symbol. The AGA 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 AGA gene?
The AGA gene provides instructions for producing an enzyme called aspartylglucosaminidase. This enzyme is active in lysosomes, which are structures inside cells that act as recycling centers. Within lysosomes, the enzyme helps break down complexes of sugar molecules (oligosaccharides) attached to certain proteins (glycoproteins). Specifically, this enzyme cuts glycoproteins between a protein building block (amino acid) called asparagine and a sugar molecule called N-acetylglucosamine. This cut is one of the last steps in breaking down a glycoprotein in the lysosome.
How are changes in the AGA gene related to health conditions?
Where is the AGA gene located?
Cytogenetic Location: 4q34.3
Molecular Location on chromosome 4: base pairs 177,430,774 to 177,442,503
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The AGA gene is located on the long (q) arm of chromosome 4 at position 34.3.
More precisely, the AGA gene is located from base pair 177,430,774 to base pair 177,442,503 on chromosome 4.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about AGA?
You and your healthcare professional may find the following resources about AGA 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 AGA 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 AGA?
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (4 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.