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Related Concept Videos

Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

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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...
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Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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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...
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Cross-bridge Cycle01:26

Cross-bridge Cycle

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As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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Formation of Muscle Fibers from Myoblasts01:13

Formation of Muscle Fibers from Myoblasts

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De novo myogenesis, or the formation of muscle fibers, begins during the early embryonic stages. The skeletal muscle is formed from somites– blocks of embryonic cell layers. The somites are further divided into dermatomes, myotomes, sclerotomes, and syndetomes. Among these, the myotomes give rise to muscle fibers.
Muscle progenitor cells (MPCs) are formed from the myotomes. MPCs express genes that encode the transcription factors Pax3 and Pax7. Along with Pax 3/7, other transcription...
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Sex-linked Disorders01:43

Sex-linked Disorders

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Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.
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Related Experiment Video

Updated: Sep 9, 2025

Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies
14:10

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

Published on: January 31, 2013

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Muscular Dystrophies.

Yi-Wen Chen1,2, Adam J Bittel3, Daniel C Bittel3

  • 1Center for Genetic Medicine Research, Children's National Research Institute, Washington, DC, USA. YChen@childrensnational.org.

Advances in Experimental Medicine and Biology
|August 29, 2025
PubMed
Summary
This summary is machine-generated.

Skeletal muscle disorders disrupt homeostasis, leading to degeneration and impaired regeneration. This chapter reviews common muscular dystrophies, their causes, mechanisms, and emerging therapies.

Keywords:
AtrophyDegenerationDystrophyMyopathyRegeneration

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Area of Science:

  • Muscle biology and regenerative medicine.
  • Pathology of neuromuscular disorders.

Background:

  • Skeletal muscles possess remarkable regenerative capacity.
  • Disruptions in molecular and cellular pathways impair muscle homeostasis.
  • Muscular dystrophies involve progressive muscle degeneration and regeneration failure.

Purpose of the Study:

  • To review genetic causes of muscular dystrophies.
  • To elucidate disease mechanisms in muscular dystrophies.
  • To discuss therapeutic developments for muscular dystrophies.

Main Methods:

  • Review of genetic causes.
  • Analysis of molecular and cellular disease mechanisms.
  • Survey of current and developing therapeutic strategies.

Main Results:

  • Identified genetic underpinnings of common muscular dystrophies.
  • Detailed molecular and cellular pathways involved in disease progression.
  • Outlined a landscape of therapeutic interventions.

Conclusions:

  • Muscular dystrophies result from complex genetic and molecular defects.
  • Regeneration failure is a key factor in muscle loss.
  • Therapeutic development offers hope for managing these progressive diseases.