Off-target autophagy inhibition by SHP2 allosteric inhibitors contributes to their antitumor activity in RAS-driven cancers

J Clin Invest. 2024 Jun 6;134(15):e177142. doi: 10.1172/JCI177142.

Abstract

Aberrant activation of RAS/MAPK signaling is common in cancer, and efforts to inhibit pathway components have yielded drugs with promising clinical activities. Unfortunately, treatment-provoked adaptive resistance mechanisms inevitably develop, limiting their therapeutic potential. As a central node essential for receptor tyrosine kinase-mediated RAS activation, SHP2 has emerged as an attractive cancer target. Consequently, many SHP2 allosteric inhibitors are now in clinical testing. Here we discovered a previously unrecognized off-target effect associated with SHP2 allosteric inhibitors. We found that these inhibitors accumulate in the lysosome and block autophagic flux in an SHP2-independent manner. We showed that off-target autophagy inhibition by SHP2 allosteric inhibitors contributes to their antitumor activity. We also demonstrated that SHP2 allosteric inhibitors harboring this off-target activity not only suppress oncogenic RAS signaling but also overcome drug resistance such as MAPK rebound and protective autophagy in response to RAS/MAPK pathway blockage. Finally, we exemplified a therapeutic framework that harnesses both the on- and off-target activities of SHP2 allosteric inhibitors for improved treatment of mutant RAS-driven and drug-resistant malignancies such as pancreatic and colorectal cancers.

Keywords: Cancer; Drug therapy; Oncology; Phosphoprotein phosphatases; Therapeutics.

MeSH terms

  • Allosteric Regulation / drug effects
  • Animals
  • Antineoplastic Agents / pharmacology
  • Antineoplastic Agents / therapeutic use
  • Autophagy* / drug effects
  • Cell Line, Tumor
  • Humans
  • MAP Kinase Signaling System / drug effects
  • Mice
  • Neoplasms / drug therapy
  • Neoplasms / enzymology
  • Neoplasms / genetics
  • Neoplasms / metabolism
  • Neoplasms / pathology
  • Protein Tyrosine Phosphatase, Non-Receptor Type 11* / antagonists & inhibitors
  • Protein Tyrosine Phosphatase, Non-Receptor Type 11* / genetics
  • Protein Tyrosine Phosphatase, Non-Receptor Type 11* / metabolism
  • Xenograft Model Antitumor Assays
  • ras Proteins* / genetics
  • ras Proteins* / metabolism

Substances

  • Antineoplastic Agents
  • Protein Tyrosine Phosphatase, Non-Receptor Type 11
  • PTPN11 protein, human
  • ras Proteins