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Targeting menin in T-lineage acute lymphoblastic leukemia - PubMed

4 hours ago
  • #menin inhibitors
  • #targeted therapy
  • #T-ALL
  • T-lineage acute lymphoblastic leukemia (T-ALL) lacks effective targeted therapies, especially for relapsed/refractory cases.
  • HOXA-high T-ALL is aggressive and often resistant to standard treatments.
  • Menin inhibitors, approved for KMT2A-rearranged leukemias, show potential efficacy in T-ALL but lack defined biomarkers.
  • Study evaluates menin inhibitors (ziftomenib, revumenib, VTP-50469) in 14 primary T-ALL samples and 8 cell lines.
  • Menin inhibitors suppressed leukemic growth in both HOXA-high and HOXA-low T-ALL samples.
  • Ziftomenib reduced tumor burden in xenografts without major toxicity.
  • Treatment down-regulated menin targets (HOXA, MEIS1, MEF2C) and upregulated T-cell differentiation programs.
  • Phosphoproteomic studies identified MEF2C S222 phosphorylation as a predictor of ziftomenib sensitivity.
  • MEF2C overexpression promoted proliferation and resistance, while knockdown impaired growth.
  • Ziftomenib synergized with CDK1/2 and ERK1/2 inhibitors, improving survival in xenografted mice.
  • High p-MEF2C S222 defines a T-ALL subset sensitive to menin inhibition.
  • Combination therapy with CDK or ERK inhibitors shows synergistic efficacy, supporting clinical trials.