CYLD gene
The CYLD gene acts as a tumor suppressor by producing an enzyme that regulates cell growth and division, helping to prevent uncontrolled cell proliferation.
Normal Function
The CYLD gene provides instructions for making an enzyme that regulates various signaling pathways involved in cell growth, including NF-KB, Wnt, JNK, TGFB1, and Notch. By managing these pathways, the CYLD enzyme ensures that cells respond appropriately to signals for growth, division (proliferation), or self-destruction (apoptosis). Because it regulates these signals, the enzyme functions as a tumor suppressor, preventing cells from growing or dividing in an uncontrolled manner.
Health Conditions Related to Genetic Changes
CYLD cutaneous syndrome
More than 100 mutations in the CYLD gene are associated with CYLD cutaneous syndrome, a condition characterized by multiple benign skin tumors, such as cylindromas, spiradenomas, and trichoepitheliomas.
- Individuals are typically born with one mutated copy of the CYLD gene, but the remaining normal copy produces enough enzyme to regulate cell growth.
- Tumors develop when a second, somatic mutation occurs in a specific cell during a person's lifetime, inactivating the remaining normal copy of the gene.
- The total loss of functional CYLD enzyme in these cells leads to uncontrolled growth and the formation of skin appendage tumors.
Cancers
Somatic mutations in the CYLD gene have been identified in various cancers, including those of the bone marrow (multiple myeloma), salivary glands, kidneys, liver, colon, and uterus. These mutations reduce CYLD gene activity, impairing its tumor suppressor function and allowing cells to divide uncontrollably and become cancerous.
Other Names for This Gene
- BRSS
- CDMT
- CYLD1
- CYLD_HUMAN
- CYLDI
- EAC
- HSPC057
- KIAA0849
- MFT
- MFT1
- SBS
- TEM
- USPL2