DHH (hedgehog)
Encyclopedia
Desert hedgehog homolog (Drosophila), also known as DHH is a human gene
Gene
A gene is a molecular unit of heredity of a living organism. It is a name given to some stretches of DNA and RNA that code for a type of protein or for an RNA chain that has a function in the organism. Living beings depend on genes, as they specify all proteins and functional RNA chains...

. The protein encoded by this gene is involved in cell signaling
Cell signaling
Cell signaling is part of a complex system of communication that governs basic cellular activities and coordinates cell actions. The ability of cells to perceive and correctly respond to their microenvironment is the basis of development, tissue repair, and immunity as well as normal tissue...

. It is named for the Desert Hedgehog
Desert Hedgehog
The desert hedgehog is a species of mammal in the Erinaceidae family.-Description:The desert hedgehog is one of the smallest of hedgehogs. It is 5.5 to 11 inches long and weighs about 10-18 ounces. The spines on its back can be banded with coloring similar to the four-toed hedgehog...

.

Function

This gene encodes a member of the Hedgehog family. The hedgehog gene family encodes signaling molecules that play an important role in regulating morphogenesis
Morphogenesis
Morphogenesis , is the biological process that causes an organism to develop its shape...

. This protein is predicted to be made as a precursor that is autocatalytically
Autocatalysis
A single chemical reaction is said to have undergone autocatalysis, or be autocatalytic, if the reaction product itself is the catalyst for that reaction....

 cleaved; the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the organism. Defects in this protein have been associated with partial gonadal dysgenesis
Gonadal dysgenesis
Gonadal dysgenesis is a term used to describe multiple reproductive system development disorders. They are conditions of genetic origin. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo....

 (PGD) accompanied by minifascicular polyneuropathy
Polyneuropathy
Polyneuropathy is a neurological disorder that occurs when many peripheral nerves throughout the body malfunction simultaneously. It may be acute and appear without warning, or chronic and develop gradually over a longer period of time. Many polyneuropathies have both motor and sensory...

. This protein may be involved in both male gonadal differentiation and perineurial
Perineurium
In the peripheral nervous system, nerve fibers are each wrapped in a protective sheath known as the endoneurium. These are bundled together into groups known as fascicles, each surrounded by a protective sheath known as the perineurium. Several fascicles may be in turn bundled together with a blood...

development.
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