zinc finger MYM-type containing 2
The ZMYM2 gene (previously known as ZNF198) provides instructions for making a protein whose function is not clearly understood. It is a member of a family of zinc finger proteins, which contain one or more short regions called zinc finger domains. The zinc finger domains in the ZMYM2 protein are thought to allow it to regulate interactions between other proteins. ZMYM2 is found in the nucleus of the cell, where it likely associates with other proteins. Through these associations, the ZMYM2 protein may be involved in repairing DNA mistakes, controlling gene activity, or forming structures in the nucleus called PML nuclear bodies that block the growth and division of cells and promote their self-destruction (apoptosis).
A genetic change involving the ZMYM2 gene causes most cases of 8p11 myeloproliferative syndrome. This condition is characterized by an increased number of white blood cells (myeloproliferative disorder) and the development of lymphoma, a blood-related cancer that causes tumor formation in the lymph nodes. The myeloproliferative disorder usually develops into another form of blood cancer called acute myeloid leukemia. 8p11 myeloproliferative syndrome most commonly results from a rearrangement (translocation) of genetic material between chromosome 13 and chromosome 8. This genetic change fuses part of the ZMYM2 gene on chromosome 13 with part of the FGFR1 gene on chromosome 8. The translocation is found only in cancer cells.
The protein produced from the normal FGFR1 gene can turn on cellular signaling that helps the cell respond to its environment, for example by stimulating cell growth. The protein produced from the fused ZMYM2-FGFR1 gene leads to constant FGFR1 signaling. The uncontrolled signaling promotes continuous cell growth and division, leading to cancer.
- fused in myeloproliferative disorders protein
- rearranged in an atypical myeloproliferative disorder
- zinc finger MYM-type protein 2
- zinc finger protein 198
- zinc finger, MYM-type 2