Desmoglein-2 deficiency drives mitochondrial morphological remodeling in cardiomyocytes

American Journal of Physiology-Heart and Circulatory Physiology 2026 Open access

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Authors: Alexandre Gonçalves, Elaine Zhelan Chen, Hosna Rastegarpouyani, Aurélia Araújo Fernandes, Inês N. Alves, Vasco Sequeira, Chulan Kwon, Stephen P. Chelko, Maicon Landim-Vieira
DOI: 10.1152/ajpheart.00368.2026
Cited by: 1
References: 127

Abstract

This study identified mitochondrial remodeling as a key feature of desmoglein-2 (DSG2)-linked ACM. Using Dsg2 mut/mut mice, we showed that DSG2 loss broadly suppresses mitochondrial transcripts involved in fusion/fission, Ca 2+ handling, mitophagy, organization, and electron transport chain assembly. Ultrastructural analyses revealed increased mitochondrial abundance, smaller and irregular mitochondrial morphology, and chamber-specific remodeling, with greater RV susceptibility. These findings support a desmosomal-mitochondrial axis and highlight mitochondrial pathways as potential therapeutic targets.

Fields of Study & Subjects

Cardiovascular Effects of Exercise Cardiac electrophysiology and arrhythmias Spinal Cord Injury Research

Publication details

Journal / source
American Journal of Physiology-Heart and Circulatory Physiology
Publisher
American Physiological Society
Volume
331
Issue
2
Pages
H440–H452
Publication date
27 June 2026
Language
EN