Search Results - "SFRH/BD/147411/2019"
Publication Catalog - "SFRH/BD/147411/2019"
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Interleukin-7 receptor α mutational activation can initiate precursor B-cell acute lymphoblastic leukemia
Publicationby Almeida, AfonsoOther Authors: Neto, João Luís; Cachucho, Ana; Euzébio, Mayara; Meng, Xiangyu; Kim, Rathana et al.Interleukin-7 receptor α (encoded by IL7R) is essential for lymphoid development. Whether acute lymphoblastic leukemia (ALL)-related IL7R gain-of-function mutations can trigger leukemogenesis remains unclear. Here, we demonstrate that lymphoid-restricted mutant IL7R, expressed at physiological levels in conditional knock-in mice, establishes a pre-leukemic stage in which B-cell precursors display self-renewal ability, initiating leukemia resembling PAX5 P80R or Ph-like human B-ALL. Full transformation associates with transcriptional upregulation of oncogenes such as Myc or Bcl2, downregulation of tumor suppressors such as Ikzf1 or Arid2, and major IL-7R signaling upregulation (involving JAK/STAT5 and PI3K/mTOR), required for leukemia cell viability. Accordingly, maximal signaling drives full penetrance and early leukemia onset in homozygous IL7R mutant animals. Notably, we identify 2 transcriptional subgroups in mouse and human Ph-like ALL, and show that dactolisib and sphingosine-kinase inhibitors are potential treatment avenues for IL-7R-related cases. Our model, a resource to explore the pathophysiology and therapeutic vulnerabilities of B-ALL, demonstrates that IL7R can initiate this malignancy.
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Page will reload when a filter is selected or excluded.- Almeida, Afonso 1 results 1
- Barata, João T. 1 results 1
- Barreto, Vasco M. 1 results 1
- Bernard-Pierrot, Isabelle 1 results 1
- Cachucho, Ana 1 results 1
- Clappier, Emannuelle 1 results 1
- Corrêa, Juliana Ronchi 1 results 1
- Duque, Mafalda 1 results 1
- Euzébio, Mayara 1 results 1
- Fernandes, Marta 1 results 1
- Fragoso, Rita 1 results 1
- Grosso, Ana R. 1 results 1
- Gu, Zhaohui 1 results 1
- Kim, Rathana 1 results 1
- Meng, Xiangyu 1 results 1
- Mullighan, Charles G. 1 results 1
- Neto, João Luís 1 results 1
- Oliveira, Mariana L. 1 results 1
- Pereira, Clara 1 results 1
- Pyne, Nigel J. 1 results 1
- Pyne, Susan 1 results 1
- Qu, Chunxu 1 results 1
- Ramos De Matos, Mafalda 1 results 1
- Raposo, Beatriz 1 results 1
- Ribeiro, Daniel 1 results 1
- Roberts, Kathryn G. 1 results 1
- Soares, Tiago 1 results 1
- Yunes, J. Andrés 1 results 1
- Zenatti, Priscila P. 1 results 1
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