Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy　– Three Decades of Progress –

Calkins, Hugh

doi:10.1253/circj.cj-15-0288

Cited by 57 publications

(65 citation statements)

References 38 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…18). Mutations in genes encoding desmosomal proteins are associated with inherited autosomal dominant diseases, such as arrhythmogenic right ventricular cardiomyopathy [reviewed in (86,248)].…”

Section: The Costamere: Protects Against Mechanical Stress and Is An mentioning

confidence: 99%

Overview of the Muscle Cytoskeleton

Henderson

Gomez

Novak

et al. 2017

Comprehensive Physiology

220

193

View full text Add to dashboard Cite

Cardiac and skeletal striated muscles are intricately designed machines responsible for muscle contraction. Coordination of the basic contractile unit, the sarcomere, and the complex cytoskeletal networks are critical for contractile activity. The sarcomere is comprised of precisely organized individual filament systems that include thin (actin), thick (myosin), titin, and nebulin. Connecting the sarcomere to other organelles (e.g., mitochondria and nucleus) and serving as the scaffold to maintain cellular integrity are the intermediate filaments. The costamere, on the other hand, tethers the sarcomere to the cell membrane. Unique structures like the intercalated disc in cardiac muscle and the myotendinous junction in skeletal muscle help synchronize and transmit force. Intense investigation has been done on many of the proteins that make up these cytoskeletal assemblies. Yet the details of their function and how they interconnect have just started to be elucidated. A vast number of human myopathies are contributed to mutations in muscle proteins; thus understanding their basic function provides a mechanistic understanding of muscle disorders. In this review, we highlight the components of striated muscle with respect to their interactions, signaling pathways, functions, and connections to disease.

show abstract

Section: The Costamere: Protects Against Mechanical Stress and Is An mentioning

confidence: 99%

Overview of the Muscle Cytoskeleton

Henderson

Gomez

Novak

et al. 2017

Comprehensive Physiology

220

193

View full text Add to dashboard Cite

show abstract

“…ARVD/C is characterized by fatty infiltration (especially within the right ventricle) and is usually caused by mutations in desmosomal proteins (Calkins, 2015). As with many cardiomyopathies, ARVD/C is also associated with an elevated risk of arrhythmia.…”

Section: Applications Of Ipsc-cms In Preclinical Screeningmentioning

confidence: 99%

Human iPSC-derived cardiomyocytes and tissue engineering strategies for disease modeling and drug screening

Smith

Macadangdang

Leung

et al. 2017

Biotechnology Advances

129

103

View full text Add to dashboard Cite

Improved methodologies for modeling cardiac disease phenotypes and accurately screening the efficacy and toxicity of potential therapeutic compounds are actively being sought to advance drug development and improve disease modeling capabilities. To that end, much recent effort has been devoted to the development of novel engineered biomimetic cardiac tissue platforms that accurately recapitulate the structure and function of the human myocardium. Within the field of cardiac engineering, induced pluripotent stem cells (iPSCs) are an exciting tool that offer the potential to advance the current state of the art, as they are derived from somatic cells, enabling the development of personalized medical strategies and patient specific disease models. Here we review different aspects of iPSC-based cardiac engineering technologies. We highlight methods for producing iPSC-derived cardiomyocytes (iPSC-CMs) and discuss their application to compound efficacy/toxicity screening and in vitro modeling of prevalent cardiac diseases. Special attention is paid to the application of micro- and nano-engineering techniques for the development of novel iPSC-CM based platforms and their potential to advance current preclinical screening modalities.

show abstract

“…The pathophysiologic basis of this disease can be dependent on defective desmosomal proteins (impaired mechanical coupling between individual cells) or intracellular signaling pathways [8]. Several genes and gene loci are associated with this disease, but cardiac magnetic resonance and signal-averaged electrocardiography are more definitive diagnostic tests than endomyocardial biopsy.…”

Section: Irreversible Cardiomyhopatiesmentioning

confidence: 99%

Introductory Chapter: Last Crossroad

Kırali¹

2017

Cardiomyopathies - Types and Treatments

View full text Add to dashboard Cite

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy　– Three Decades of Progress –

Cited by 57 publications

References 38 publications

Overview of the Muscle Cytoskeleton

Overview of the Muscle Cytoskeleton

Human iPSC-derived cardiomyocytes and tissue engineering strategies for disease modeling and drug screening

Introductory Chapter: Last Crossroad

Contact Info

Product

Resources

About

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy – Three Decades of Progress –

Cited by 57 publications

References 38 publications

Overview of the Muscle Cytoskeleton

Overview of the Muscle Cytoskeleton

Human iPSC-derived cardiomyocytes and tissue engineering strategies for disease modeling and drug screening

Introductory Chapter: Last Crossroad

Contact Info

Product

Resources

About

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy　– Three Decades of Progress –