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Review
. 2016 May 18:4:49.
doi: 10.3389/fped.2016.00049. eCollection 2016.

The Functional Role of PRC2 in Early T-cell Precursor Acute Lymphoblastic Leukemia (ETP-ALL) - Mechanisms and Opportunities

Kathrin M Bernt  1 , Stephen P Hunger  2 , Tobias Neff  1
Affiliations
Review

The Functional Role of PRC2 in Early T-cell Precursor Acute Lymphoblastic Leukemia (ETP-ALL) - Mechanisms and Opportunities

Kathrin M Bernt et al. Front Pediatr. .

Abstract

Early T-Cell precursor acute lymphoblastic leukemia (ETP-ALL) is a relatively newly identified subset of T-lineage ALL. There are conflicting results regarding prognosis, and the genetic basis of this condition is variable. Here, we summarize the current status of the field and discuss the role of mutations in the Polycomb Repressive Complex 2 frequently identified in ETP-ALL patients.

Keywords: Hox genes; JAK/STAT signaling pathway; epigenetics; leukemia; lymphoid; polycomb repressive complex.

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Figures

Figure 1
Figure 1
Cellular consequences of compromised PRC2 function in ETP-ALL. Inactivation of PRC2 components in ETP-ALL results in cellular loss of global cellular H3K27me3, a chromatin mark associated with silent genes. A subset of these genes show increased transcription. Noteworthy groups of target genes with increased expression in Ras-transformed cells with compromised PRC2 function include transcription factors and epigenetic regulators associated with early hematopoiesis (e.g., HoxA, Gata2, and Bmi1) and growth factors and their receptors (e.g., Il6ra).
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