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Reviewed September 2010
What is the official name of the SHH gene?
The official name of this gene is “sonic hedgehog.”
SHH is the gene's official symbol. The SHH 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 SHH gene?
The SHH gene provides instructions for making a protein called Sonic Hedgehog. This protein functions as a chemical signal that is essential for embryonic development. Sonic Hedgehog plays a role in cell growth, cell specialization, and the normal shaping (patterning) of the body. This protein is important for development of the brain and spinal cord (central nervous system), eyes, limbs, and many other parts of the body.
Sonic Hedgehog is necessary for the development of the front part of the brain (forebrain). This signaling protein helps establish the line that separates the right and left sides of the forebrain (the midline). Specifically, Sonic Hedgehog establishes the midline for the underside (ventral surface) of the forebrain. Sonic Hedgehog and other signaling proteins are needed to form the right and left halves (hemispheres) of the brain.
Sonic Hedgehog also has an important role in the formation of the eyes. During early development, the cells that develop into the eyes form a single structure called the eye field. This structure is located in the center of the developing face. Sonic hedgehog signaling causes the eye field to separate into two distinct eyes.
How are changes in the SHH gene related to health conditions?
Genetics Home Reference provides information about these additional conditions, which are also associated with changes in the SHH gene:
Where is the SHH gene located?
Cytogenetic Location: 7q36
Molecular Location on chromosome 7: base pairs 155,595,557 to 155,604,966
The SHH gene is located on the long (q) arm of chromosome 7 at position 36.
More precisely, the SHH gene is located from base pair 155,595,557 to base pair 155,604,966 on chromosome 7.
See How do geneticists indicate the location of a gene? in the Handbook.
Where can I find additional information about SHH?
You and your healthcare professional may find the following resources about SHH 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 SHH 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 SHH?
You may find definitions for these and many other terms in the Genetics Home Reference Glossary.
See also Understanding Medical Terminology.
References (10 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.