Research Papers:

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 and Geertruy te Kronnie

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Oncotarget. 2017; 8:42398-42413. https://doi.org/10.18632/oncotarget.16392

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Elena Vendramini1,2,3, Marco Giordan1, Emanuela Giarin1, Barbara Michielotto1, Grazia Fazio4, Gianni Cazzaniga4, Andrea Biondi4, Daniela Silvestri4, Maria Grazia Valsecchi5, Martina U. Muckenthaler6, Andreas E. Kulozik6, Valter Gattei7, Shai Izraeli2,3, Giuseppe Basso1 and Geertruy te Kronnie1

1Department of Women’s and Children’s Health, University of Padova, Padova, Italy

2Edmond and Lily Safra Children’s Hospital, Sheba Medical Center, Ramat Gan, Israel

3Tel Aviv University, Tel Aviv, Israel

4Centro Ricerca Tettamanti, Clinica Pediatrica, University of Milano-Bicocca, Monza, Italy

5School of Medicine and Surgery, University of Milano-Bicocca, Milano, Italy

6Department of Pediatric Oncology Hematology, University of Heidelberg, Heidelberg, Germany

7Clinical and Experimental Onco-Hematology Unit, Centro di Riferimento Oncologico, I.R.C.C.S., Aviano (PN), Italy

Correspondence to:

Elena Vendramini, email: [email protected]

Keywords: B cell precursor acute lymphoblastic leukemia, ERG aberrations, noncoding RNAs, miR-125, SNORD116

Received: June 29, 2016    Accepted: March 04, 2017    Published: March 21, 2017


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.

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