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Genetics Home Reference: your guide to understanding genetic conditions
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ZMYM2

Reviewed July 2013

What is the official name of the ZMYM2 gene?

The official name of this gene is “zinc finger, MYM-type 2.”

ZMYM2 is the gene's official symbol. The ZMYM2 gene is also known by other names, listed below.

What is the normal function of the ZMYM2 gene?

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).

Does the ZMYM2 gene share characteristics with other genes?

The ZMYM2 gene belongs to a family of genes called ZMYM (zinc fingers, MYM type).

A gene family is a group of genes that share important characteristics. Classifying individual genes into families helps researchers describe how genes are related to each other. For more information, see What are gene families? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genefamilies) in the Handbook.

How are changes in the ZMYM2 gene related to health conditions?

8p11 myeloproliferative syndrome - caused by mutations in the ZMYM2 gene

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.

Where is the ZMYM2 gene located?

Cytogenetic Location: 13q11-q12

Molecular Location on chromosome 13: base pairs 19,957,567 to 20,091,828

The ZMYM2 gene is located on the long (q) arm of chromosome 13 between positions 11 and 12.

The ZMYM2 gene is located on the long (q) arm of chromosome 13 between positions 11 and 12.

More precisely, the ZMYM2 gene is located from base pair 19,957,567 to base pair 20,091,828 on chromosome 13.

See How do geneticists indicate the location of a gene? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genelocation) in the Handbook.

Where can I find additional information about ZMYM2?

You and your healthcare professional may find the following resources about ZMYM2 helpful.

You may also be interested in these resources, which are designed for genetics professionals and researchers.

What other names do people use for the ZMYM2 gene or gene products?

  • 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
  • ZMYM2_HUMAN
  • ZNF198

See How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What glossary definitions help with understanding ZMYM2?

acute ; acute myeloid leukemia ; apoptosis ; atypical ; cancer ; cell ; chromosome ; DNA ; gene ; leukemia ; lymph ; lymphoma ; myeloid ; nucleus ; protein ; rearrangement ; syndrome ; translocation ; tumor ; white blood cells

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://ghr.nlm.nih.gov/glossary).

References

  • Gocke CB, Yu H. ZNF198 stabilizes the LSD1-CoREST-HDAC1 complex on chromatin through its MYM-type zinc fingers. PLoS One. 2008 Sep 22;3(9):e3255. doi: 10.1371/journal.pone.0003255. (http://www.ncbi.nlm.nih.gov/pubmed/18806873?dopt=Abstract)
  • Jackson CC, Medeiros LJ, Miranda RN. 8p11 myeloproliferative syndrome: a review. Hum Pathol. 2010 Apr;41(4):461-76. doi: 10.1016/j.humpath.2009.11.003. Review. (http://www.ncbi.nlm.nih.gov/pubmed/20226962?dopt=Abstract)
  • Kunapuli P, Kasyapa CS, Chin SF, Caldas C, Cowell JK. ZNF198, a zinc finger protein rearranged in myeloproliferative disease, localizes to the PML nuclear bodies and interacts with SUMO-1 and PML. Exp Cell Res. 2006 Nov 15;312(19):3739-51. Epub 2006 Aug 14. (http://www.ncbi.nlm.nih.gov/pubmed/17027752?dopt=Abstract)
  • Kunapuli P, Somerville R, Still IH, Cowell JK. ZNF198 protein, involved in rearrangement in myeloproliferative disease, forms complexes with the DNA repair-associated HHR6A/6B and RAD18 proteins. Oncogene. 2003 May 29;22(22):3417-23. (http://www.ncbi.nlm.nih.gov/pubmed/12776193?dopt=Abstract)
  • NCBI Gene (http://www.ncbi.nlm.nih.gov/gene/7750)
  • Xiao S, McCarthy JG, Aster JC, Fletcher JA. ZNF198-FGFR1 transforming activity depends on a novel proline-rich ZNF198 oligomerization domain. Blood. 2000 Jul 15;96(2):699-704. (http://www.ncbi.nlm.nih.gov/pubmed/10887137?dopt=Abstract)
  • Xiao S, Nalabolu SR, Aster JC, Ma J, Abruzzo L, Jaffe ES, Stone R, Weissman SM, Hudson TJ, Fletcher JA. FGFR1 is fused with a novel zinc-finger gene, ZNF198, in the t(8;13) leukaemia/lymphoma syndrome. Nat Genet. 1998 Jan;18(1):84-7. (http://www.ncbi.nlm.nih.gov/pubmed/9425908?dopt=Abstract)
  • OMIM: ZINC FINGER, MYM-TYPE 2 (http://omim.org/entry/602221)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: July 2013
Published: October 20, 2014