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

Cellular Dynamics of Transgenic Porcine Endothelial Cells to Inflammatory Stimuli in Xenotransplantation Settings.

Xenotransplantation·2026

Same author

Aortic asprosin overexpression does not ameliorate disease pathophysiology in a murine model of Marfan syndrome.

Scientific reports·2026

Same author

Targeting Inflammation by Pioglitazone and its R-Enantiomer Mitigates Pathological Myocardial Remodeling in Murine Hypertrophic Cardiomyopathy.

JACC. Basic to translational science·2026

Same author

A retrospective study of morbidity and mortality in captive Przewalski's horses (Equus przewalskii) between 1973 and 2021.

Journal of comparative pathology·2026

Same author

Algorithm-based quantification of tissue vascularization in immunohistochemical stainings of tissue sections.

Scientific reports·2026

Same author

Borna Disease Virus 1 as Cause of Fatal Meningoencephalomyelitis in Wild Hedgehogs, Germany, 2022-2025.

Emerging infectious diseases·2026

Search research articles

Related Experiment Video

Updated: Jan 16, 2026

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

Published on: January 31, 2013

Reduced Muscle Force in Dystrophic DMDΔ52 Pigs Is Incompletely Restored by Systemic Transcript Reframing (DMDΔ51-52).

Michaela Blasi^1,2, Hristiyan Hristov^1,2, Jan B Stöckl³

¹Chair of Molecular Animal Breeding and Biotechnology, Gene Center and Department of Veterinary Sciences, LMU Munich, Munich, Germany.

Journal of Cachexia, Sarcopenia and Muscle

|October 1, 2025

Summary

This summary is machine-generated.

Duchenne muscular dystrophy (DMD) pigs with exon 51 skipping partially restored muscle function, but force generation did not reach wild-type (WT) levels. This study highlights dynamic muscle force measurements as a key tool for evaluating therapies in DMD models.

Keywords:

Becker muscular dystrophy Duchenne muscular dystrophy dystrophin muscle force pig model

More Related Videos

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors

Published on: September 14, 2019

Assessing Functional Performance in the Mdx Mouse Model

Assessing Functional Performance in the Mdx Mouse Model

Published on: March 27, 2014

Related Experiment Videos

Last Updated: Jan 16, 2026

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

Published on: January 31, 2013

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors

Published on: September 14, 2019

Assessing Functional Performance in the Mdx Mouse Model

Assessing Functional Performance in the Mdx Mouse Model

Published on: March 27, 2014

Area of Science:

Biomedical Sciences
Genetics and Genomics
Animal Models

Background:

Duchenne muscular dystrophy (DMD) is a severe X-linked genetic disorder causing progressive muscle degeneration.
DMD pigs lacking exon 52 (DMDΔ52) serve as a severe DMD model.
Becker muscular dystrophy (BMD) pigs (DMDΔ51-52) model a milder form of the disease.

Purpose of the Study:

To quantitatively assess skeletal muscle function in dystrophic pigs.
To evaluate the efficacy of exon 51 skipping in restoring muscle function in DMD models.
To establish dynamic muscle force measurements as a tool for therapeutic evaluation.

Main Methods:

Utilized the Aurora Swine Isometric Footplate Test Apparatus for hind limb dorsiflexor muscle analysis.
Quantified functional parameters in DMD pigs, BMD pigs, and wild-type (WT) controls.
Conducted histopathological and proteomic analyses of muscle tissues.

Main Results:

DMD and BMD muscles showed partial restoration of peak force (62.4% and 67.1% of WT, respectively) after twitch stimulation.
Normalized force values were 70.9% for DMD and 65.8% for BMD pigs.
Muscle contraction and relaxation rates were altered in both DMD and BMD pigs compared to WT, with more severe changes in DMD pigs.

Conclusions:

Systemic exon 51 skipping in DMD pigs partially restores skeletal muscle function but does not fully recover WT levels.
Dynamic muscle force measurements are a sensitive indicator for assessing therapeutic interventions in porcine DMD models.
Significant muscle pathology was observed in DMD pigs, while BMD pigs showed minimal differences compared to WT controls.