NF-κB inhibition rescues cardiac function by remodeling calcium genes in a Duchenne muscular dystrophy model

Nat Commun. 2018 Aug 24;9(1):3431. doi: 10.1038/s41467-018-05910-1.

Abstract

Duchenne muscular dystrophy (DMD) is a neuromuscular disorder causing progressive muscle degeneration. Although cardiomyopathy is a leading mortality cause in DMD patients, the mechanisms underlying heart failure are not well understood. Previously, we showed that NF-κB exacerbates DMD skeletal muscle pathology by promoting inflammation and impairing new muscle growth. Here, we show that NF-κB is activated in murine dystrophic (mdx) hearts, and that cardiomyocyte ablation of NF-κB rescues cardiac function. This physiological improvement is associated with a signature of upregulated calcium genes, coinciding with global enrichment of permissive H3K27 acetylation chromatin marks and depletion of the transcriptional repressors CCCTC-binding factor, SIN3 transcription regulator family member A, and histone deacetylase 1. In this respect, in DMD hearts, NF-κB acts differently from its established role as a transcriptional activator, instead promoting global changes in the chromatin landscape to regulate calcium genes and cardiac function.

Publication types

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

MeSH terms

  • Animals
  • CCCTC-Binding Factor / metabolism
  • Calcium / metabolism
  • Cells, Cultured
  • Chromatin Assembly and Disassembly / genetics
  • Chromatin Assembly and Disassembly / physiology
  • Histone Deacetylase 1 / genetics
  • Histone Deacetylase 1 / metabolism
  • Male
  • Mice
  • Mice, Inbred mdx
  • Muscular Dystrophy, Duchenne / genetics
  • Muscular Dystrophy, Duchenne / metabolism*
  • Myocytes, Cardiac / metabolism*
  • NF-kappa B / metabolism*
  • Repressor Proteins / genetics
  • Repressor Proteins / metabolism
  • Signal Transduction / physiology
  • Sin3 Histone Deacetylase and Corepressor Complex
  • Sodium-Calcium Exchanger / genetics
  • Sodium-Calcium Exchanger / metabolism

Substances

  • CCCTC-Binding Factor
  • NF-kappa B
  • Repressor Proteins
  • SIN3A transcription factor
  • Sodium-Calcium Exchanger
  • sodium-calcium exchanger 1
  • Histone Deacetylase 1
  • Sin3 Histone Deacetylase and Corepressor Complex
  • Calcium