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Related Concept Videos

Satellite Stem Cells and Muscular Dystrophy01:21

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...
Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin01:26

Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin

Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
Skeletal Muscle Relaxants: Therapeutic Uses01:31

Skeletal Muscle Relaxants: Therapeutic Uses

Skeletal muscle relaxants are used to relax muscle tone and alleviate painful muscle contractions. However, the choice of skeletal muscle relaxants depends on the duration of the surgical procedure in order to minimize potential side effects. Skeletal muscle relaxants like neuromuscular blocking agents [NMBAs] are commonly employed as adjuvants alongside general anesthetics in clinical settings. NMBAs are also used to maintain controlled ventilation during surgery of the larynx or pharynx as...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...

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Related Experiment Video

Updated: May 8, 2026

CRISPR/Cas9 Technology in Restoring Dystrophin Expression in iPSC-Derived Muscle Progenitors
07:44

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

Published on: September 14, 2019

Therapeutic advances in muscular dystrophy.

Doris G Leung1, Kathryn R Wagner

  • 1Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, MD; Departments of Neurology, Johns Hopkins School of Medicine, Baltimore, MD.

Annals of Neurology
|August 14, 2013
PubMed
Summary

Advances in understanding genetic muscular dystrophies are leading to new therapies. This review covers Duchenne, facioscapulohumeral, and myotonic dystrophy treatments, offering hope for improved quality of life.

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Area of Science:

  • Neurology
  • Genetics
  • Pharmacology

Background:

  • Muscular dystrophies are genetic disorders causing progressive muscle weakness.
  • Recent scientific advances are driving new therapeutic strategies and clinical trials.

Purpose of the Study:

  • To review therapeutic developments for Duchenne muscular dystrophy, facioscapulohumeral muscular dystrophy, and myotonic dystrophy.
  • To highlight the diverse molecular mechanisms and therapeutic approaches for different genetic muscular dystrophy classes.

Main Methods:

  • Review of current scientific literature on muscular dystrophy.
  • Analysis of therapeutic developments across monogenic, epigenetic, and repeat expansion disorders.

Main Results:

  • Significant progress in understanding muscular dystrophy pathogenesis.
  • Numerous novel pharmacologic agents are in development for these conditions.

Conclusions:

  • Therapeutic strategies are tailored to the specific genetic and molecular basis of each muscular dystrophy.
  • Emerging treatments show promise for improving the quality of life for individuals with muscular dystrophy.