Expression Quantitative Trait Locus Analysis in Systemic Sclerosis Identifies New Candidate Genes Associated With Multiple Aspects of Disease Pathology

Arthritis Rheumatol. 2021 Jul;73(7):1288-1300. doi: 10.1002/art.41657. Epub 2021 May 28.

Abstract

Objective: To identify the genetic variants that affect gene expression (expression quantitative trait loci [eQTLs]) in systemic sclerosis (SSc) and to investigate their role in the pathogenesis of the disease.

Methods: We performed an eQTL analysis using whole-blood sequencing data from 333 SSc patients and 524 controls and integrated them with SSc genome-wide association study (GWAS) data. We integrated our findings from expression modeling, differential expression analysis, and transcription factor binding site enrichment with key clinical features of SSc.

Results: We detected 49,123 validated cis-eQTLs from 4,539 SSc-associated single-nucleotide polymorphisms (SNPs) (PGWAS < 10-5 ). A total of 1,436 genes were within 1 Mb of the 4,539 SSc-associated SNPs. Of those 1,436 genes, 565 were detected as having ≥1 eQTL with an SSc-associated SNP. We developed a strategy to prioritize disease-associated genes based on their expression variance explained by SSc eQTLs (r2 > 0.05). As a result, 233 candidates were identified, 134 (58%) of them associated with hallmarks of SSc and 105 (45%) of them differentially expressed in the blood cells, skin, or lung tissue of SSc patients. Transcription factor binding site analysis revealed enriched motifs of 24 transcription factors (5%) among SSc eQTLs, 5 of which were found to be differentially regulated in the blood cells (ELF1 and MGA), skin (KLF4 and ID4), and lungs (TBX4) of SSc patients. Ten candidate genes (4%) can be targeted by approved medications for immune-mediated diseases, of which only 3 have been tested in clinical trials in patients with SSc.

Conclusion: The findings of the present study indicate a new layer to the molecular complexity of SSc, contributing to a better understanding of the pathogenesis of the disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Basic Helix-Loop-Helix Transcription Factors / genetics
  • Female
  • Gene Expression Regulation / genetics*
  • Genetic Association Studies
  • Humans
  • Inhibitor of Differentiation Proteins / genetics
  • Kruppel-Like Factor 4
  • Kruppel-Like Transcription Factors / genetics
  • Male
  • Middle Aged
  • Molecular Targeted Therapy
  • Nuclear Proteins / genetics
  • Polymorphism, Single Nucleotide
  • Quantitative Trait Loci
  • Scleroderma, Systemic / genetics*
  • T-Box Domain Proteins / genetics
  • Transcription Factors / genetics

Substances

  • Basic Helix-Loop-Helix Transcription Factors
  • ELF1 protein, human
  • ID4 protein, human
  • Inhibitor of Differentiation Proteins
  • KLF4 protein, human
  • Kruppel-Like Factor 4
  • Kruppel-Like Transcription Factors
  • MGA protein, human
  • Nuclear Proteins
  • T-Box Domain Proteins
  • TBX4 protein, human
  • Transcription Factors