The unfolded protein response impacts melanoma progression by enhancing FGF expression and can be antagonized by a chemical chaperone

Sci Rep. 2017 Dec 13;7(1):17498. doi: 10.1038/s41598-017-17888-9.

Abstract

The mechanisms hallmarking melanoma progression are insufficiently understood. Here we studied the impact of the unfolded protein response (UPR) - a signalling cascade playing ambiguous roles in carcinogenesis - in melanoma malignancy. We identified isogenic patient-derived melanoma cell lines harboring BRAFV600E-mutations as a model system to study the role of intrinsic UPR in melanoma progression. We show that the activity of the three effector pathways of the UPR (ATF6, PERK and IRE1) was increased in metastatic compared to non-metastatic cells. Increased UPR-activity was associated with increased flexibility to cope with ER stress. The activity of the ATF6- and the PERK-, but not the IRE-pathway, correlated with poor survival in melanoma patients. Using whole-genome expression analysis, we show that the UPR is an inducer of FGF1 and FGF2 expression and cell migration. Antagonization of the UPR using the chemical chaperone 4-phenylbutyric acid (4-PBA) reduced FGF expression and inhibited cell migration and viability. Consistently, FGF expression positively correlated with the activity of ATF6 and PERK in human melanomas. We conclude that chronic UPR stimulates the FGF/FGF-receptor signalling axis and promotes melanoma progression. Hence, the development of potent chemical chaperones to antagonize the UPR might be a therapeutic approach to target melanoma.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antineoplastic Agents / pharmacology*
  • Biomarkers, Tumor / metabolism
  • Butylamines / pharmacology*
  • Cell Line, Tumor
  • Cell Movement / physiology
  • Cell Survival / drug effects
  • Cell Survival / physiology
  • Disease Progression
  • Endoplasmic Reticulum Stress / physiology
  • Fibroblast Growth Factors / metabolism*
  • Gene Expression Regulation, Neoplastic / drug effects
  • Gene Expression Regulation, Neoplastic / physiology
  • Humans
  • Melanocytes / drug effects
  • Melanocytes / metabolism
  • Melanoma / drug therapy*
  • Melanoma / genetics
  • Melanoma / metabolism*
  • Mice
  • Mutation
  • Neoplasm Transplantation
  • Proto-Oncogene Proteins B-raf / genetics
  • Proto-Oncogene Proteins B-raf / metabolism
  • Unfolded Protein Response / drug effects*

Substances

  • 4-phenylbutylamine
  • Antineoplastic Agents
  • Biomarkers, Tumor
  • Butylamines
  • Fibroblast Growth Factors
  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf