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International Journal of Hematology
Published in: International Journal of Hematology 2/2019

01-08-2019 | Primary Myelofibrosis | Progress in Hematology

Deregulated Polycomb functions in myeloproliferative neoplasms

Authors: Goro Sashida, Motohiko Oshima, Atsushi Iwama

Published in: International Journal of Hematology | Issue 2/2019

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Abstract

Polycomb proteins function in the maintenance of gene silencing via post-translational modifications of histones and chromatin compaction. Genetic and biochemical studies have revealed that the repressive function of Polycomb repressive complexes (PRCs) in transcription is counteracted by the activating function of Trithorax-group complexes; this balance fine-tunes the expression of genes critical for development and tissue homeostasis. The function of PRCs is frequently dysregulated in various cancer cells due to altered expression or recurrent somatic mutations in PRC genes. The tumor suppressive functions of EZH2-containing PRC2 and a PRC2-related protein ASXL1 have been investigated extensively in the pathogenesis of hematological malignancies, including myeloproliferative neoplasms (MPN). BCOR, a component of non-canonical PRC1, suppresses various hematological malignancies including MPN. In this review, we focus on recent findings on the role of PRCs in the pathogenesis of MPN and the therapeutic impact of targeting the pathological functions of PRCs in MPN.
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Metadata
Title
Deregulated Polycomb functions in myeloproliferative neoplasms
Authors
Goro Sashida
Motohiko Oshima
Atsushi Iwama
Publication date
01-08-2019
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 2/2019
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-019-02600-6

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