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Reviewed February 2010

What is the official name of the NAGA gene?

The official name of this gene is “N-acetylgalactosaminidase, alpha-.”

NAGA is the gene's official symbol. The NAGA 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 NAGA gene?

The NAGA gene provides instructions for making the enzyme alpha-N-acetylgalactosaminidase. This enzyme works in the lysosomes, which are compartments within cells that digest and recycle materials. Within lysosomes, the enzyme helps break down complexes called glycoproteins and glycolipids, which consist of sugar molecules attached to certain proteins and fats. Specifically, alpha-N-acetylgalactosaminidase helps remove a molecule called alpha-N-acetylgalactosamine from sugars in these complexes.

How are changes in the NAGA gene related to health conditions?

Schindler disease - caused by mutations in the NAGA gene

Approximately seven NAGA gene mutations have been identified in people with Schindler disease. Most of these mutations are believed to change the 3-dimensional shape of the alpha-N-acetylgalactosaminidase enzyme, interfering with its ability to break down glycoproteins and glycolipids. These substances accumulate in the lysosomes and cause cells to malfunction and eventually die. Cell damage in the various tissues and organs of the body leads to neurological problems and the other signs and symptoms of Schindler disease.

Where is the NAGA gene located?

Cytogenetic Location: 22q11

Molecular Location on chromosome 22: base pairs 42,058,334 to 42,070,871

(Homo sapiens Annotation Release 107, GRCh38.p2) (NCBIThis link leads to a site outside Genetics Home Reference.)

The NAGA gene is located on the long (q) arm of chromosome 22 at position 11.

The NAGA gene is located on the long (q) arm of chromosome 22 at position 11.

More precisely, the NAGA gene is located from base pair 42,058,334 to base pair 42,070,871 on chromosome 22.

See How do geneticists indicate the location of a gene? in the Handbook.

Where can I find additional information about NAGA?

You and your healthcare professional may find the following resources about NAGA 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 NAGA gene or gene products?

  • Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B)
  • alpha-N-acetylgalactosaminidase
  • alpha-N-acetylgalactosaminidase precursor
  • D22S674
  • GALB

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 NAGA?

cell ; enzyme ; gene ; glycolipids ; glycoproteins ; molecule ; neurological ; precursor

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.

Reviewed: February 2010
Published: February 1, 2016