Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Satellite Stem Cells and Muscular Dystrophy

Satellite Stem Cells and Muscular Dystrophy

Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

RBM20 variants disrupt Ca<sup>2+</sup> handling and metabolism in dilated and non-compaction cardiomyopathy stem cell models.

Signal transduction and targeted therapy·2026

Same author

Mutant SOD1 expressed by oligodendrocytes aggregates in myelinic nanochannels and accelerates disease progression in familial ALS mice.

bioRxiv : the preprint server for biology·2026

Same author

Oligodendrocyte Piezo2 is a regulator of age-dependent myelin integrity and dysregulated in multiple sclerosis.

Communications biology·2026

Same author

Targeting intracellular cholesterol imbalance rescues sarcomere-ER contact site signaling and ER remodeling in dilated cardiomyopathy.

Signal transduction and targeted therapy·2026

Same author

Left atrial appendage occluder thrombosis caused embolic strokes: a case report.

European heart journal. Case reports·2026

Same author

Stem-Cell-Derived Biologic Ventricular Assist Tissue in Heart Failure.

The New England journal of medicine·2026

Search research articles

Related Experiment Video

Updated: Jun 21, 2025

DNA Electroporation, Isolation and Imaging of Myofibers

DNA Electroporation, Isolation and Imaging of Myofibers

Published on: December 23, 2015

Dysferlin Enables Tubular Membrane Proliferation in Cardiac Hypertrophy.

Nora Josefine Paulke^1,2, Carolin Fleischhacker^1,2, Justus B Wegener^1,2

¹Department of Cardiology and Pneumology (N.J.P., C.F., J.B.W., G.C.R., M.M., N.Z., Y.Z., J.W., L.L., A.A.G., D.K.-D., E.W., T.K., K.T., G.H., S.E.L., S.B.), University Medical Center Göttingen, Germany.

Circulation Research

|July 16, 2024

Summary

Dysferlin is crucial for maintaining cardiomyocyte structure during heart hypertrophy. Its absence impairs the transverse-axial tubule network, leading to reduced cardiac function and increased risk of heart failure.

Keywords:

cardiomyopathy, dilated dysferlin hypertrophy, left ventricular intracellular membranes membrane fusion proteomics ryanodine receptor calcium release channel

More Related Videos

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

Simultaneous Electrical and Mechanical Stimulation to Enhance Cells' Cardiomyogenic Potential

Simultaneous Electrical and Mechanical Stimulation to Enhance Cells' Cardiomyogenic Potential

Published on: January 18, 2019

Related Experiment Videos

Last Updated: Jun 21, 2025

DNA Electroporation, Isolation and Imaging of Myofibers

DNA Electroporation, Isolation and Imaging of Myofibers

Published on: December 23, 2015

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

Simultaneous Electrical and Mechanical Stimulation to Enhance Cells' Cardiomyogenic Potential

Simultaneous Electrical and Mechanical Stimulation to Enhance Cells' Cardiomyogenic Potential

Published on: January 18, 2019

Area of Science:

Cardiovascular Biology
Cellular Cardiology
Membrane Biology

Background:

Cardiac hypertrophy is a response to heart stress but can lead to heart failure.
The transverse-axial tubule (TAT) network is vital for cardiomyocyte function.
Dysferlin's role in cardiac TAT network integrity and hypertrophy was unexplored.

Purpose of the Study:

To investigate the role of dysferlin in maintaining cardiomyocyte TAT network integrity.
To determine dysferlin's contribution to TAT endomembrane proliferation during cardiac hypertrophy.

Main Methods:

Localization of dysferlin in cardiomyocytes using super-resolution microscopy.
Analysis of the cardiac dysferlin interactome and proteomic changes in knockout mice.
Assessment of cardiac function and TAT network remodeling in response to pressure overload.

Main Results:

Dysferlin localizes to the sarcoplasmic reticulum-cardiomyocyte membrane junctions.
Dysferlin interacts with juncophilin-2, a key component of excitation-contraction coupling.
Dysferlin knockout mice showed reduced hypertrophy and impaired TAT network reorganization.
Increased dysferlin expression correlated with TAT network proliferation in wild-type hearts.

Conclusions:

Dysferlin is essential for protecting junctional complexes and regulating TAT network reorganization.
Dysferlin controls tubular membrane proliferation in cardiomyocytes during pressure overload.
Dysferlin emerges as a novel therapeutic target for cardiac disease.