PANoptosis is a prominent feature of desmoplakin cardiomyopathy.

ABSTRACT:

Introduction

Arrhythmogenic cardiomyopathy (ACM) is hereditary cardiomyopathy caused by pathogenic variants (mutations) in genes encoding the intercalated disc (ID), particularly desmosome proteins. ACM caused by mutations in the DSP gene encoding desmoplakin (DSP) is characterized by the prominence of cell death, myocardial fibrosis, and inflammation, and is referred to as desmoplakin cardiomyopathy.

Aim

The aim of this article was to gain insight into the pathogenesis of DSP cardiomyopathy.

Methods and results

The Dsp gene was exclusively deleted in cardiac myocytes using tamoxifen-inducible MerCreMer (Myh6-Mcm ^Tam) and floxed Dsp (Dsp ^F/F) mice (Myh6-Mcm ^Tam:Dsp ^F/F). Recom

SUBMITTER: Olcum M 

PROVIDER: S-EPMC9933912 | biostudies-literature | 2023 Feb

REPOSITORIES: biostudies-literature