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...

Full description

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

Similar Items

Aberrant Mitochondrial Fission Is Maladaptive in Desmin Mutation–Induced Cardiac Proteotoxicity
by: Shafiul Alam, et al.
Published: (2018-07-01)

Pleiotropic effects of mdivi-1 in altering mitochondrial dynamics, respiration, and autophagy in cardiomyocytes
by: Richa Aishwarya, et al.
Published: (2020-09-01)

Molecular Perspectives of Mitochondrial Adaptations and Their Role in Cardiac Proteostasis
by: Shafiul Alam, et al.
Published: (2020-08-01)

Desminopathy: Novel Desmin Variants, a New Cardiac Phenotype, and Further Evidence for Secondary Mitochondrial Dysfunction
by: Miloš Kubánek, et al.
Published: (2020-03-01)

Desmin common mutation is associated with multi-systemic disease manifestations and depletion of mitochondria and mitochondrial DNA
by: Elizabeth eMcCormick, et al.
Published: (2015-06-01)

Desmin Interacts Directly with Mitochondria
by: Alexander A. Dayal, et al.
Published: (2020-10-01)

Desmin Knock-Out Cardiomyopathy: A Heart on the Verge of Metabolic Crisis
by: Barbara Elsnicova, et al.
Published: (2022-10-01)

Mitochondrial dysfunction and autophagy activation are associated with cardiomyopathy developed by extended methamphetamine self-administration in rats
by: Chowdhury S. Abdullah, et al.
Published: (2022-12-01)

Desmin: molecular interactions and putative functions of the muscle intermediate filament protein
by: M.L. Costa, et al.
Published: (2004-12-01)

Electrophilic Aldehyde 4-Hydroxy-2-Nonenal Mediated Signaling and Mitochondrial Dysfunction
by: Sudha Sharma, et al.
Published: (2022-10-01)

Desmin-related restrictive cardiomyopathy in a pediatric patient: A case report
by: Shruti Sharma, et al.
Published: (2013-01-01)

Is Desmin Propensity to Aggregate Part of its Protective Function?
by: Sonia R. Singh, et al.
Published: (2020-02-01)

Unraveling Desmin’s Head Domain Structure and Function
by: Dimitrios Vlachakis, et al.
Published: (2024-03-01)

Desmin variants: Trigger for cardiac arrhythmias?
by: Wei Su, et al.
Published: (2022-09-01)

Deep Characterization of a Greek Patient with Desmin-Related Myofibrillar Myopathy and Cardiomyopathy
by: Constantinos Papadopoulos, et al.
Published: (2023-07-01)

Features of myocardial injury detected by cardiac magnetic resonance in a patient with desmin‐related restrictive cardiomyopathy
by: Zixian Chen, et al.
Published: (2021-12-01)

Critical contribution of mitochondria in the development of cardiomyopathy linked to desmin mutation
by: Yeranuhi Hovhannisyan, et al.
Published: (2024-01-01)

Novel Desmin Mutation Causing Myofibrillar Myopathy in a Hmong Family
by: Stefan Nicolau, et al.
Published: (2020-01-01)

Desmin-related cardiomyopathy presenting as restrictive cardiomyopathy: A case report with review of literature
by: Kalpana Kumari, et al.
Published: (2016-01-01)

Cardiac Dysfunction in the Sigma 1 Receptor Knockout Mouse Associated With Impaired Mitochondrial Dynamics and Bioenergetics
by: Chowdhury S. Abdullah, et al.
Published: (2018-10-01)

Case report: An unusual case of desmin myopathy associated with heart failure and arrhythmia
by: Xuhan Liu, et al.
Published: (2022-07-01)

Distribution and Expression of Vimentin and Desmin in Broiler Pectoralis major Affected by the Growth-Related Muscular Abnormalities
by: Francesca Soglia, et al.
Published: (2020-01-01)

Metformin Protects against Diabetic Cardiomyopathy: An Association between Desmin–Sarcomere Injury and the iNOS/mTOR/TIMP-1 Fibrosis Axis
by: Amal F. Dawood, et al.
Published: (2022-04-01)

Changes in cell shape and desmin intermediate filament distribution are associated with down-regulation of desmin expression in C2C12 myoblasts grown in the absence of extracellular Ca2+
by: C.S. Mermelstein, et al.
Published: (2005-07-01)

Corrigendum: Case report: An unusual case of desmin myopathy associated with heart failure and arrhythmia
by: Xuhan Liu, et al.
Published: (2022-10-01)

Editorial: Mitochondrial Dysfunction in Stroke
by: Feng Yan, et al.
Published: (2022-03-01)

Desmin and dystrophin abnormalities in upper airway muscles of snorers and patients with sleep apnea
by: Farhan Shah, et al.
Published: (2019-02-01)

Temperature Changes During and After Eccentric Contractions and Its Effect on Force and Desmin Loss in Rat
by: Javad Mosayebnejad, et al.
Published: (2011-04-01)

Desmin expression in colorectal cancer stroma correlates with advanced stage disease and marks angiogenic microvessels
by: Arentz Georgia, et al.
Published: (2011-12-01)

Host Desmin Interacts with RABV Matrix Protein and Facilitates Virus Propagation
by: Wen Zhang, et al.
Published: (2023-02-01)

Lack of Desmin in Mice Causes Structural and Functional Disorders of Neuromuscular Junctions
by: Nane Eiber, et al.
Published: (2020-10-01)

Ultrastructural Evidence of Mitochondrial Dysfunction in Osteomyelitis Patients
by: Daniel H. Mendelsohn, et al.
Published: (2023-03-01)

Cardiac-specific inactivation of LPP3 in mice leads to myocardial dysfunction and heart failure
by: Mini Chandra, et al.
Published: (2018-04-01)

The Association of Tau With Mitochondrial Dysfunction in Alzheimer's Disease
by: Ying Cheng, et al.
Published: (2018-03-01)

Mitochondrial Dysfunction in Individuals with Diabetic Kidney Disease: A Systematic Review
by: Nicole Flemming, et al.
Published: (2022-08-01)

Mitochondrial biology and dysfunction in secondary mitochondrial disease
by: Megan J. Baker, et al.
Published: (2022-12-01)

Mitochondrial protein dysfunction in pathogenesis of neurological diseases
by: Liang Wang, et al.
Published: (2022-09-01)

Advances in exercise to alleviate sarcopenia in older adults by improving mitochondrial dysfunction
by: Yang Zhu, et al.
Published: (2023-07-01)

A case report of adolescent myofibrillar myopathy due to a de novo R406W pathogenic variant in desmin with symptoms of “hypertrophic cardiomyopathy”
by: Hongyan Xiao, et al.
Published: (2024-02-01)

Woven bone formation and mineralization by rat mesenchymal stromal cells imply increased expression of the intermediate filament desmin
by: Giusy Di Conza, et al.
Published: (2023-09-01)