A Therapeutically Targetable NOTCH1–SIRT1–KAT7 Axis in T-cell Leukemia

Olga Lancho, Amartya Singh, Victoria da Silva-Diz, Maya Aleksandrova, Jesminara Khatun, Luca Tottone, Patricia Renck Nunes, Shirley Luo, Caifeng Zhao, Haiyan Zheng, Eric Chiles, Zhenyu Zuo, Pedro P. Rocha, Xiaoyang Su, Hossein Khiabanian, Daniel Herranz

Research output: Contribution to journalArticlepeer-review

Abstract

T-cell acute lymphoblastic leukemia (T-ALL) is a NOTCH1-driven disease in need of novel therapies. Here, we identify a NOTCH1–SIRT1–KAT7 link as a therapeutic vulnerability in T-ALL, in which the histone deacetylase SIRT1 is overexpressed downstream of a NOTCH1-bound enhancer. SIRT1 loss impaired leukemia generation, whereas SIRT1 overexpression accelerated leukemia and conferred resistance to NOTCH1 inhibition in a deacetylase-dependent manner. Moreover, pharmacologic or genetic inhibition of SIRT1 resulted in significant antileukemic effects. Global acetyl proteomics upon SIRT1 loss uncovered hyperacetylation of KAT7 and BRD1, subunits of a histone acetyltransferase complex targeting H4K12. Metabolic and gene-expression profiling revealed metabolic changes together with a transcriptional signature resembling KAT7 deletion. Consistently, SIRT1 loss resulted in reduced H4K12ac, and overexpression of a nonacetylatable KAT7-mutant partly rescued SIRT1 loss-induced proliferation defects. Overall, our results uncover therapeutic targets in T-ALL and reveal a circular feedback mechanism balancing deacetylase/acetyltransferase activation with potentially broad relevance in cancer. SIGNIFICANCE: We identify a T-ALL axis whereby NOTCH1 activates SIRT1 through an enhancer region, and SIRT1 deacetylates and activates KAT7. Targeting SIRT1 shows antileukemic effects, partly mediated by KAT7 inactivation. Our results reveal T-ALL therapeutic targets and uncover a rheostat mechanism between deacetylase/acetyltransferase activities with potentially broader cancer relevance.

Original languageEnglish (US)
Pages (from-to)13-33
Number of pages21
JournalBlood cancer discovery
Volume4
Issue number1
DOIs
StatePublished - Jan 1 2023

All Science Journal Classification (ASJC) codes

  • General Medicine

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