Therapeutic Modulation of the Immune Response in Arrhythmogenic Cardiomyopathy.

ABSTRACT:

Background

Inflammation is a prominent feature of arrhythmogenic cardiomyopathy (ACM), but whether it contributes to the disease phenotype is not known.

Methods

To define the role of inflammation in the pathogenesis of ACM, we characterized nuclear factor-κB signaling in ACM models in vitro and in vivo and in cardiac myocytes from patient induced pluripotent stem cells.

Results

Activation of nuclear factor-κB signaling, indicated by increased expression and nuclear accumulation of phospho-RelA/p65, occurred in both an in vitro model of ACM (expression of JUP^2157del2 in neonatal rat ventricular myocytes) and a robust murine model of ACM (homozygous knock-in of mutant desmoglein-2 [Dsg2^mut/mut]) that recapitulates the cardiac m

SUBMITTER: Chelko SP 

PROVIDER: S-EPMC6817418 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature