The ribosomal DNA genes (rDNA) exist at approximately 300-400 copies in mammalian cells, and their transcription is highly upregulated in cancers. CX-5461 is a specific inhibitor of RNA polymerase I (Pol I) mediated rDNA transcription and is currently in phase I clinical trial for hematological malignancies. Specifically, inhibition of rDNA transcription by CX-5461 leads to activation of both p53-dependent and -independent cellular checkpoints1,2. Further, activation of a DNA damage response (DDR) via ATM and ATR signaling are essential for CX-5461’s ability to arrest leukemia cells in S and G2/M phases (Hein et al., under review). Importantly, factors involved in DDR have been shown to greatly sensitise cancer cells to CX-5461 treatment2. It is known that mechanistic interactions underlying the DDR rely on context-specific chromatin structure, including the structural rearrangement of the chromatin by histone chaperones and the bromodomain and extra-terminal family of proteins (BET)3. Due to the highly specialised transcriptional context of rDNA, we hypothesise that targeting epigenetic and transcriptional regulators in combination with CX-5461 may render cancer cells highly sensitive to ATM/ATR activation and result in enhanced anti-tumour activity.
In this study we utilised IBET-151, an inhibitor that blocks BET protein binding to acetylated chromatin marks in combination with CX-5461 in a disease model of acute myeloid leukemia (AML). We observed a range of synergy between CX-5461 and IBET-151 in a panel of human AML cell lines. Importantly, co-treatment with CX-5461 and IBET-151 significantly improved survival over single agents alone in a preclinical model of aggressive MLL-AF9; NrasG12D-driven AML. Studies in human AML cell lines showed an increase in rDNA accessibility to micrococcal nuclease digestion following treatment with IBET-151 or the knockdown of its target, BRD4. We propose that inhibition of BRD4 by IBET-151 results in an ‘open’ state of rDNA chromatin which leads to an enhanced CX-5461-mediated DDR. This model is supported by enhanced checkpoint activation, including a heightened γH2AX response following combined CX-5461 and IBET-151 treatment. We will present our work thus far investigating the mechanistic role of this drug combination in eliciting synergistic checkpoint activation in AML