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Oncoscience | A murine model for human early/immature T-cell precursor acute lymphoblastic leukemia (EITP ALL)

Figure 1 (truncated): Idh2R140Q/NHD13 double transgenic mice develop EITP ALL resembling the human disease. Credit: 2022 Negi and Aplan

A new research perspective titled "A murine model for human early/immature T-cell precursor acute lymphoblastic leukemia (EITP ALL)" has been published in Oncoscience.

In this research perspective, researchers Vijay Negi and Peter D. Aplan from the National Institutes of Health's National Cancer Institute discuss early/immature T cell precursor acute lymphoblastic leukemia (EITP ALL). EITP ALL represents a subset of human leukemias distinct from other T-ALL, and associated with poor prognosis. Clinical studies have identified chromosomal translocations involving the NUP98 gene and point mutations of IDH genes as recurrent mutations in patients with EITP-ALL.

"In a recent study using genetically engineered mice, we demonstrated that cooperation of an Idh2R140Q mutation with a NUP98-HOXD13 (NHD13) fusion gene resulted in EITP-ALL," the researchers write.

Highlights of this double transgenic mouse model include the similarity of the immunophenotypic, mutational and gene expression landscape with human EITP-ALL. Additional studies showed that the Idh2R140Q/NHD13 EITP-ALL are sensitive to selective mutant IDH2 inhibitors in vitro, leading to the possibility that these mice can serve as a useful model for the study of EITP ALL development and therapy.

"We predict that the Idh2R140Q/NHD13 mouse model will serve as an excellent tool to study EITP biology and identify therapies for patients with EITP leukemia," conclude the researchers.

More information: Vijay Negi et al, A murine model for human early/immature T-cell precursor acute lymphoblastic leukemia (EITP ALL), Oncoscience (2022). DOI: 10.18632/oncoscience.567

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