• What is TET2?
  • TET2 in Myelodysplastic Syndromes
  • Clinical Trials

TET2

Tet methylcytosine dioxygenase 2 (TET2; also known as ten-eleven translocation 2) is a gene that codes for a protein involved in epigenetic regulation of myelopoeisis (Gene 2014; Solary et al. 2014). TET2 is a tumor suppressor, and so in cancer, loss of TET2 function, which can occur via TET2 mutation, TET2 deletion, or IDH1 or IDH2 mutation, can cause myeloid or lymphoid transformations (Solary et al. 2014). Mutations in TET2 have been found in MDS, AML, ALL, and other hematologic malignancies.​

Related Pathways

Contributors: Stephen A. Strickland, M.D., MSCI, Annette S. Kim, M.D., Ph.D.

Suggested Citation: Strickland, S., A. Kim. 2015. TET2. My Cancer Genome https://www.padiracinnovation.org/content/disease/myelodysplastic-syndromes/tet2/?tab=0 (Updated December 4).

Last Updated: December 4, 2015

TET2 in Myelodysplastic Syndromes

TET2 mutations occur in 18.7% of MDS (COSMIC). TET2 mutations are observed in all types of MDS, and they tend to co-occur with SRFS2 in chronic myelomonocytic leukemia (CMML), a subtype of MDS (Cazzola, Della Porta, and Malcovati 2013). TET2 mutations are believed to cause loss of function (Solary et al. 2014). TET2 is a tumor suppressor gene, and so loss-of-function mutations support the abnormal hematopoiesis observed in MDS (Solary et al. 2014). These mutations are found spread throughout the gene.

TET2 mutations are a neutral or favorable prognostic biomarker (Bejar et al. 2011; Kosminder et al. 2009; NCCN 2014). However, Bejar et al. (2014) observed that TET2 mutations predict shorter overall survival following hematopoietic stem cell transplantation.​

Contributors: Stephen A. Strickland, M.D., MSCI, Annette S. Kim, M.D., Ph.D.

Suggested Citation: Strickland, S., A. Kim. 2014. TET2 in Myelodysplastic Syndromes. My Cancer Genome https://www.padiracinnovation.org/content/disease/myelodysplastic-syndromes/tet2/ (Updated September 23).

Last Updated: September 23, 2014

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