Autosomal Recessive Cardiomyopathy Presenting as Acute Myocarditis.

ABSTRACT:

Background

Myocarditis is inflammation of the heart muscle that can follow various viral infections. Why children only rarely develop life-threatening acute viral myocarditis (AVM), given that the causal viral infections are common, is unknown. Genetic lesions might underlie such susceptibilities. Mouse genetic studies demonstrated that interferon (IFN)-α/β immunity defects increased susceptibility to virus-induced myocarditis. Moreover, variations in human TLR3, a potent inducer of IFNs, were proposed to underlie AVM.

Objectives

This study sought to evaluate the hypothesis that human genetic factors may underlie AVM in previously healthy children.

Methods

We tested the role of TLR3-IFN immunity using human induced pluripotent stem cell-derived cardiomyocytes. We then

SUBMITTER: Belkaya S 

PROVIDER: S-EPMC5551973 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature