Targeted degradation of MDM2 overcomes feedback regulation of p53 signaling in Merkel cell carcinoma models

MDM2 is transcriptionally activated by the ST-MYCL-Tip60 complex in virus-positive Merkel cell carcinoma (MCC). MDM2 suppresses p53 and is a rational therapeutic target. MDM2 inhibitors face an intrinsic limitation: p53 activation induces MDM2 transcription, creating a feedback loop that blunts inhibitor efficacy. We demonstrate that MDM2 degraders KTX-049 and KT-253 overcome this limitation by collapsing the p53/MDM2 negative feedback loop. KTX-049 was >100-fold more potent than the MDM2 inhibitor DS-3032 across WT p53 MCC cell lines, and this superior potency was quantitatively supported by mechanistic mathematical modeling. In vivo, KT-253 produced deep and durable tumor regressions, including complete responses in patient-derived xenograft models. Acquired resistance was strongly associated with acquisition of TP53 mutations, confirming on-target pathway pressure. These findings establish feedback architecture as a critical determinant of therapeutic response and position MDM2 degradation as a qualitatively distinct strategy that produces more durable pathway engagement than MDM2 inhibition, providing a preclinical rationale for prioritizing MDM2 degraders in WT TP53 MCC.

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Publication Details

Journal
Journal of Clinical Investigation
Published
2026-06-30
DOI
https://doi.org/10.1172/jci199049
Primary Topic
Polyomavirus and related diseases
Type
article
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article

Targeted degradation of MDM2 overcomes feedback regulation of p53 signaling in Merkel cell carcinoma models

C Howarth, Varsha Ananthapadmanabhan, Benjamin K. Eschle, João A. Paulo et al.
Journal of Clinical Investigation
Polyomavirus and related diseases
article

Targeted degradation of MDM2 overcomes feedback regulation of p53 signaling in Merkel cell carcinoma models

C Howarth, Varsha Ananthapadmanabhan, Benjamin K. Eschle, João A. Paulo, Andrew D. Cherniack, Joana S. Rodrigues, Susanne B. Breitkopf, Prafulla C. Gokhale, James A. DeCaprio, Joyoti Dey, Franziska Michor, Rujuta Kshirsagar, Abeba Teshager, Leonard Vonk, Yvonne Li, Julia Schnabel, Yogesh Chutake, Kirti Sharma, Yu-Chen Cheng, Ananya Kodali, Simone Bruno
article en

Abstract

MDM2 is transcriptionally activated by the ST-MYCL-Tip60 complex in virus-positive Merkel cell carcinoma (MCC). MDM2 suppresses p53 and is a rational therapeutic target. MDM2 inhibitors face an intrinsic limitation: p53 activation induces MDM2 transcription, creating a feedback loop that blunts inhibitor efficacy. We demonstrate that MDM2 degraders KTX-049 and KT-253 overcome this limitation by collapsing the p53/MDM2 negative feedback loop. KTX-049 was >100-fold more potent than the MDM2 inhibitor DS-3032 across WT p53 MCC cell lines, and this superior potency was quantitatively supported by mechanistic mathematical modeling. In vivo, KT-253 produced deep and durable tumor regressions, including complete responses in patient-derived xenograft models. Acquired resistance was strongly associated with acquisition of TP53 mutations, confirming on-target pathway pressure. These findings establish feedback architecture as a critical determinant of therapeutic response and position MDM2 degradation as a qualitatively distinct strategy that produces more durable pathway engagement than MDM2 inhibition, providing a preclinical rationale for prioritizing MDM2 degraders in WT TP53 MCC.

Journal of Clinical InvestigationVol. 136(13)
Broad Institute (US), Brigham and Women's Hospital (US), Harvard University (US), Harvard University Press (US), Woomera Therapeutics (US), Dana-Farber Cancer Institute (US)
Openalex Percentile: Top 9%
Polyomavirus and related diseases
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