Dysfunctional Mitochondrial Dynamic and Oxidative Phosphorylation Precedes Cardiac Dysfunction in R120G‐αB‐Crystallin‐Induced Desmin‐Related Cardiomyopathy

Dysfunctional Mitochondrial Dynamic and Oxidative Phosphorylation Precedes Cardiac Dysfunction in R120G‐αB‐Crystallin‐Induced Desmin‐Related Cardiomyopathy

Background The mutated α‐B‐Crystallin (CryABR120G) mouse model of desmin‐related myopathy (DRM) shows an age‐dependent onset of pathologic cardiac remodeling and progression of heart failure. CryABR120G expression in cardiomyocytes affects the mitochondrial spatial organization within the myofibrils...

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Bibliographic Details
Main Authors: Shafiul Alam, Chowdhury S. Abdullah, Richa Aishwarya, Mahboob Morshed, Sadia S. Nitu, Sumitra Miriyala, Manikandan Panchatcharam, Christopher G. Kevil, A. Wayne Orr, Md. Shenuarin Bhuiyan
Format: Article
Language:English
Published: Wiley 2020-12-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
desmin‐related myopathy
mitochondrial dynamics
mitochondrial respiration
oxidative phosphorylation
R120G‐αB‐crystallin
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.120.017195

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https://www.ahajournals.org/doi/10.1161/JAHA.120.017195

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