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.
Authors
- C Howarth
- Varsha Ananthapadmanabhan (ORCID: https://orcid.org/0000-0002-6909-1460)
- Benjamin K. Eschle
- João A. Paulo (ORCID: https://orcid.org/0000-0002-4291-413X)
- Andrew D. Cherniack (ORCID: https://orcid.org/0000-0003-0470-0111)
- Joana S. Rodrigues (ORCID: https://orcid.org/0000-0001-7800-1204)
- Susanne B. Breitkopf
- Prafulla C. Gokhale (ORCID: https://orcid.org/0000-0002-1974-5921)
- James A. DeCaprio (ORCID: https://orcid.org/0000-0002-0896-167X)
- Joyoti Dey
- Franziska Michor (ORCID: https://orcid.org/0009-0003-8552-7767)
- Rujuta Kshirsagar
- Abeba Teshager
- Leonard Vonk
- Yvonne Li (ORCID: https://orcid.org/0000-0002-1741-1995)
- Julia Schnabel
- Yogesh Chutake
- Kirti Sharma
- Yu-Chen Cheng
- Ananya Kodali
- Simone Bruno
Institutions
- Broad Institute (US)
- Brigham and Women's Hospital (US)
- Harvard University (US)
- Harvard University Press (US)
- Woomera Therapeutics (US)
- Dana-Farber Cancer Institute (US)
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
- Field-Weighted Citation Impact
- 0.00