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Reviewed August 2010
What is the official name of the SGSH gene?
The official name of this gene is “N-sulfoglucosamine sulfohydrolase.”
SGSH is the gene's official symbol. The SGSH 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 SGSH gene?
The SGSH gene provides instructions for producing an enzyme called sulfamidase. This enzyme is located in lysosomes, compartments within cells that digest and recycle different types of molecules. Sulfamidase is involved in the step-wise breakdown of large molecules called glycosaminoglycans (GAGs). GAGs are composed of sugar molecules that are linked together to form a long string. To break down these large molecules, individual sugars are removed one at a time from one end of the molecule. Sulfamidase removes a chemical group known as a sulfate from a sugar called glucosamine when it is at the end of the GAG chain.
How are changes in the SGSH gene related to health conditions?
Where is the SGSH gene located?
Cytogenetic Location: 17q25.3
Molecular Location on chromosome 17: base pairs 80,206,966 to 80,220,400
(Homo sapiens Annotation Release 107, GRCh38.p2) (
The SGSH gene is located on the long (q) arm of chromosome 17 at position 25.3.
More precisely, the SGSH gene is located from base pair 80,206,966 to base pair 80,220,400 on chromosome 17.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about SGSH?
You and your healthcare professional may find the following resources about SGSH 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 SGSH 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 SGSH?
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.