A hotspot mutation in transcription factor IKZF3 drives B cell neoplasia via transcriptional dysregulation.

doi:10.1016/j.ccell.2021.02.003

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Article

A hotspot mutation in transcription factor IKZF3 drives B cell neoplasia via transcriptional dysregulation.

Cancer cell - 8 Mar 2021

Lazarian Gregory, Yin Shanye, Ten Hacken Elisa, Sewastianik Tomasz, Uduman Mohamed, Font-Tello Alba, Gohil Satyen H, Li Shuqiang, Kim Ekaterina, Joyal Heather, Billington Leah, Witten Elizabeth, Zheng Mei, Huang Teddy, Severgnini Mariano, Lefebvre Valerie, Rassenti Laura Z, Gutierrez Catherine, Georgopoulos Katia, Ott Christopher J, Wang Lili, Kipps Thomas J, Burger Jan A, Livak Kenneth J, Neuberg Donna S, Baran-Marszak Fanny, Cymbalista Florence, Carrasco Ruben D, Wu Catherine J

Abstract excerpt

Hotspot mutation of IKZF3 (IKZF3-L162R) has been identified as a putative driver of chronic lymphocytic leukemia (CLL), but its function remains unknown. Here, we demonstrate its driving role in CLL through a B cell-restricted conditional knockin mouse model. Mutant Ikzf3 alters DNA binding specificity and target selection, leading to hyperactivation of B cell receptor (BCR) signaling, overexpression of nuclear...

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PMID: 33689703
DOI: 10.1016/j.ccell.2021.02.003
PMCID: PMC8034546

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