Targeting E3 Ubiquitin Ligases and Deubiquitinases in Ciliopathy and Cancer

Int J Mol Sci. 2020 Aug 19;21(17):5962. doi: 10.3390/ijms21175962.

Abstract

Cilia are antenna-like structures present in many vertebrate cells. These organelles detect extracellular cues, transduce signals into the cell, and play an essential role in ensuring correct cell proliferation, migration, and differentiation in a spatiotemporal manner. Not surprisingly, dysregulation of cilia can cause various diseases, including cancer and ciliopathies, which are complex disorders caused by mutations in genes regulating ciliary function. The structure and function of cilia are dynamically regulated through various mechanisms, among which E3 ubiquitin ligases and deubiquitinases play crucial roles. These enzymes regulate the degradation and stabilization of ciliary proteins through the ubiquitin-proteasome system. In this review, we briefly highlight the role of cilia in ciliopathy and cancer; describe the roles of E3 ubiquitin ligases and deubiquitinases in ciliogenesis, ciliopathy, and cancer; and highlight some of the E3 ubiquitin ligases and deubiquitinases that are potential therapeutic targets for these disorders.

Keywords: cancer; cilia; ciliogenesis; ciliopathy; differentiation; proliferation; ubiquitin–proteasome pathway.

Publication types

  • Review

MeSH terms

  • Animals
  • Antineoplastic Agents / pharmacology
  • Antineoplastic Agents / therapeutic use
  • Ciliopathies / drug therapy*
  • Ciliopathies / metabolism
  • Deubiquitinating Enzymes / metabolism*
  • Enzyme Inhibitors / pharmacology
  • Enzyme Inhibitors / therapeutic use
  • Humans
  • Neoplasms / drug therapy*
  • Neoplasms / metabolism
  • Ubiquitin-Protein Ligases / metabolism*
  • Ubiquitination / drug effects

Substances

  • Antineoplastic Agents
  • Enzyme Inhibitors
  • Ubiquitin-Protein Ligases
  • Deubiquitinating Enzymes

Grants and funding