DCC gene
The DCC gene provides instructions for making the netrin-1 receptor, a protein essential for nervous system development and tumor suppression.
Normal Function
The DCC gene provides instructions for making a protein called the netrin-1 receptor, which is involved in the development of the nervous system. This protein attaches (binds) to a protein called netrin-1, and these two proteins fit together like a lock and key. The binding of netrin-1 to its receptor helps direct the growth of specialized nerve cell extensions called axons. Axons transmit nerve impulses that can signal muscle movement.
When the brain sends a signal to trigger muscle movement, the signal usually travels from one half of the brain to the opposite side of the body. The binding of netrin-1 to its receptor allows axons to develop this signaling pathway.
The netrin-1 receptor is also thought to act as a dependence receptor, which means it functions differently depending on whether or not it is bound to its protein. When the netrin-1 receptor is bound to netrin-1, it plays a role in nervous system development. When it is not bound to netrin-1, the netrin-1 receptor acts as a tumor suppressor, which means that it keeps cells from growing and dividing too fast or in an uncontrolled way. When the netrin-1 receptor is acting as a tumor suppressor, studies suggest that the netrin-1 receptor triggers the self-destruction of cells when they are no longer needed (apoptosis).
Health Conditions Related to Genetic Changes
Congenital mirror movement disorder
DCC gene variants (also called mutations) have been identified in people with congenital mirror movement disorder, a condition in which intentional movements of one side of the body are mirrored by involuntary movements of the other side of the body. These variants change single protein building blocks (amino acids) in the netrin-1 receptor or cause cells to produce an abnormally short version of the protein. Without functional netrin-1 proteins, the growth of axons during nervous system development is not properly directed. As a result, movement signals that originate in one half of the brain are transmitted to both sides of the body, leading to mirror movements.
Cancers
The loss (deletion) of the DCC gene and nearby genetic material has been found in the majority of colorectal cancer cases. This deletion is also found in other cancerous tumors but not as frequently. In the cancer cases that have been associated with the loss of the DCC gene, the deletion was not inherited and was only found in the tumor cells. Without the DCC gene, cells cannot produce the netrin-1 receptor. As a result, the netrin-1 receptor is not available to trigger apoptosis, resulting in the uncontrolled cell growth and division that leads to cancer.
Other Names for This Gene
- CRC18
- CRCR1
- DCC_HUMAN
- IGDCC1
- MRMV1
- netrin receptor DCC
- NTN1R1
- tumor suppressor protein DCC