High expression of miR-125b-2 and SNORD116 noncoding RNA clusters characterize ERG-related B cell precursor acute lymphoblastic leukemia

Elena Vendramini*, Marco Giordan, Emanuela Giarin, Barbara Michielotto, Grazia Fazio, Gianni Cazzaniga, Andrea Biondi, Daniela Silvestri, Maria Grazia Valsecchi, Martina U. Muckenthaler, Andreas E. Kulozik, Valter Gattei, Shai Izraeli, Giuseppe Basso, Geertruy te Kronnie

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

19 Scopus citations

Abstract

ERG-related leukemia is a B cell precursor acute lymphoblastic leukemia (BCP ALL) subtype characterized by aberrant expression of DUX4 and ERG transcription factors, and highly recurrent ERG intragenic deletions. ERG-related patients have remarkably favorable outcome despite a high incidence of inauspicious IKZF1 aberrations. We describe clinical and genomic features of the ERG-related cases in an unselected cohort of B-other BCP ALL pediatric patients enrolled in the AIEOP ALL 2000 therapeutic protocol. We report a small noncoding RNA signature specific of ERG-related group, with up-regulation of miR-125b-2 cluster on chromosome 21 and several snoRNAs in the Prader-Willi locus at 15q11.2, including the orphan SNORD116 cluster.

Original languageEnglish
Pages (from-to)42398-42413
Number of pages16
JournalOncotarget
Volume8
Issue number26
DOIs
StatePublished - 2017

Funding

FundersFunder number
Seventh Framework Programme261474

    Keywords

    • B cell precursor acute lymphoblastic leukemia
    • ERG aberrations
    • MiR-125
    • Noncoding RNAs
    • SNORD116

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