Disruption of KEAP1/CUL3/RBX1 E3-ubiquitin ligase complex components by multiple genetic mechanisms: Association with poor prognosis in head and neck cancer

Head Neck. 2015 May;37(5):727-34. doi: 10.1002/hed.23663. Epub 2014 Jun 18.

Abstract

Background: The NRF2 pathway has multiple pro-tumorigenic functions, and Nrf2 levels are increased in head and neck squamous cell carcinoma (HNSCC). The KEAP1/CUL3/RBX1 E3-ubiquitin ligase complex is a negative regulator of NRF2. In this study, we investigated mechanisms of disruption of individual complex components.

Methods: Clinical and genomic profiles for 302 patients with HNSCC were obtained from The Cancer Genome Atlas. Combined pattern of epi/genetic alterations for individual components revealed frequent of complex disruption. Gene-set enrichment analysis was performed on expression data to identify affected pathways.

Results: DNA loss is the main mechanism of alteration for all component genes, whereas hypermethylation largely affects only KEAP1. Combined analysis revealed that 64% of patients with HNSCC have disruption in this protein complex. Concordantly, NRF2-associated gene signature is enriched in HNSCC. Survival was significantly diminished among patients with one or more disrupted components.

Conclusion: The KEAP1/CUL3/RBX1 E3-ubiquitin ligase complex is frequently disrupted in HNSCC by multiple mechanisms. NRF2-based prognostics will benefit from integrated analysis of component genes.

Keywords: KEAP1/CUL3/RBX1 E3-ubiquitin ligase complex; NRF2; gene disruption; head and neck squamous cell carcinoma (HNSCC); survival.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Carcinoma, Squamous Cell / genetics*
  • Carcinoma, Squamous Cell / mortality*
  • Carcinoma, Squamous Cell / therapy
  • Carrier Proteins / genetics
  • Cullin Proteins / genetics
  • Databases, Factual
  • Female
  • Gene Expression Regulation, Neoplastic*
  • Head and Neck Neoplasms / genetics*
  • Head and Neck Neoplasms / mortality*
  • Head and Neck Neoplasms / therapy
  • Humans
  • Intracellular Signaling Peptides and Proteins / genetics
  • Kaplan-Meier Estimate
  • Kelch-Like ECH-Associated Protein 1
  • Male
  • NF-E2-Related Factor 2 / genetics
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies
  • Signal Transduction
  • Survival Analysis
  • Ubiquitin-Protein Ligase Complexes / genetics*

Substances

  • CUL3 protein, human
  • Carrier Proteins
  • Cullin Proteins
  • Intracellular Signaling Peptides and Proteins
  • KEAP1 protein, human
  • Kelch-Like ECH-Associated Protein 1
  • NF-E2-Related Factor 2
  • NFE2L2 protein, human
  • RBX1 protein, human
  • Ubiquitin-Protein Ligase Complexes