Current Hematologic Malignancy Reports

, Volume 7, Issue 1, pp 57–64

Role of TET2 Mutations in Myeloproliferative Neoplasms

Authors

  • Elodie Pronier
    • Institut National de la Santé et de la Recherche Médicale, UMR 1009, Institut Gustave RoussyUniversité Paris Sud (Paris 11)
    • Université Denis Diderot (Paris 7)
    • Université Pierre et Marie Curie (Paris 6) Laboratoire d’Hématologie, Hôpital Saint-Antoine
Myeloproliferative Neoplasms (JJ Kiladjian, Section Editor)

DOI: 10.1007/s11899-011-0108-8

Cite this article as:
Pronier, E. & Delhommeau, F. Curr Hematol Malig Rep (2012) 7: 57. doi:10.1007/s11899-011-0108-8

Abstract

Recently, 5-hydroxymethylcytosine (5-hmC), the 6th base of DNA, was discovered as the product of the hydroxylation of 5-methylcytosine (5-mC) by the ten-eleven translocation (TET) oncogene family members. One of them, TET oncogene family member 2 (TET2), is mutated in a variety of myeloid malignancies, including in 15% of myeloproliferative neoplasms (MPNs). Recent studies tried to go further into the biological and epigenetic function of TET2 protein and 5-hmC marks in the pathogenesis of myeloid malignancies. Although its precise function remains partially unknown, TET2 appears to be an important regulator of hematopoietic stem cell biology. In both mouse and human cells, its inactivation leads to a dramatic deregulation of hematopoiesis that ultimately triggers blood malignancies. Understanding this leukemogenic process will provide tools to develop new epigenetic therapies against blood cancers.

Keywords

TET2Mutations5-hydroxymethylcytosineMyeloproliferative neoplasmsMyeloid malignanciesHematopoietic stem cellsEpigeneticsPathogenesis

Copyright information

© Springer Science+Business Media, LLC 2011