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ZMYM2 gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

ZMYM2 gene

The ZMYM2 gene provides instructions for a zinc finger protein that likely regulates protein interactions and plays a role in DNA repair and cell cycle control.

Normal Function

The ZMYM2 gene (previously known as ZNF198) provides instructions for making a protein that is a member of the zinc finger family. These proteins contain regions called zinc finger domains, which are thought to allow the ZMYM2 protein to regulate interactions between other proteins.

Located in the nucleus, ZMYM2 likely associates with other proteins to perform several functions, including:

  • Repairing DNA errors
  • Controlling gene activity
  • Forming structures called PML nuclear bodies, which block cell growth and division and promote self-destruction (apoptosis)

Health Conditions Related to Genetic Changes

8p11 myeloproliferative syndrome

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 and the development of lymphoma, which often progresses to acute myeloid leukemia.

The syndrome typically results from a translocation of genetic material between chromosome 13 and chromosome 8, which fuses part of the ZMYM2 gene with the FGFR1 gene. This fusion creates a protein that leads to constant, uncontrolled FGFR1 signaling, which promotes continuous cell growth and division, resulting in cancer.

Other Names for This Gene

  • FIM
  • fused in myeloproliferative disorders protein
  • MYM
  • RAMP
  • rearranged in an atypical myeloproliferative disorder
  • SCLL
  • zinc finger MYM-type protein 2
  • zinc finger protein 198
  • zinc finger, MYM-type 2
  • ZMYM2_HUMAN
  • ZNF198

Frequently Asked Questions

The fusion creates a protein that continuously activates FGFR1 signaling, driving uncontrolled growth of white blood cells. Symptoms include unexplained high white blood cell counts, fatigue, fever, weight loss, or signs of lymphoma such as swollen lymph nodes. Persistent or unexplained leukocytosis should prompt a hematology referral.
Diagnostic confirmation uses cytogenetic analysis (karyotyping) or fluorescence in situ hybridization (FISH) to detect the translocation t(8;13). Molecular methods like reverse‑transcription PCR or next‑generation sequencing can also identify the ZMYM2‑FGFR1 fusion transcript.
Standard therapy often involves intensive chemotherapy followed by allogeneic stem‑cell transplantation, which offers the best chance for long‑term remission. Targeted FGFR inhibitors (e.g., ponatinib) have shown promise in early studies but are not yet universally approved for this specific fusion. Treatment plans should be individualized and discussed with a specialized oncology team.
Because the translocation is typically a somatic event rather than inherited, routine screening of family members is not recommended. However, if a family history of myeloproliferative disorders exists, genetic counseling can help assess risk and discuss surveillance strategies.===
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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