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Related Concept Videos

Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

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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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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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Alterations in Muscle Tone ll01:12

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Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
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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

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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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Related Experiment Video

Updated: May 5, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
09:39

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Published on: July 29, 2016

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[Myotonic dystrophy].

Shoichi Ishiura1, Kosuke Oana, Michinori Koebis

  • 1Graduate School of Arts and Sciences, The University of Tokyo.

Rinsho Shinkeigaku = Clinical Neurology
|December 3, 2013
PubMed
Summary

Researchers developed novel antisense oligonucleotide delivery and small molecule compounds to treat myotonic dystrophy. These approaches successfully increased chloride channel protein expression and corrected gene splicing in mouse models, offering new therapeutic avenues.

Area of Science:

  • Biomedical science
  • Molecular biology
  • Neuromuscular disorders

Background:

  • Myotonic dystrophy lacks effective treatments, posing a significant challenge in clinical management.
  • Current therapeutic strategies for myotonic dystrophy are limited, necessitating the development of novel approaches.

Purpose of the Study:

  • To establish an effective antisense oligonucleotide delivery system for skeletal muscle in myotonic dystrophy.
  • To identify small molecule compounds capable of correcting aberrant Clcn1 gene splicing.

Main Methods:

  • Utilized bubble liposomes for antisense oligonucleotide delivery to the skeletal muscle of myotonic dystrophy mouse models.
  • Screened small molecule compounds for their ability to correct Clcn1 gene splicing defects.

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Myo-mechanical Analysis of Isolated Skeletal Muscle
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Related Experiment Videos

Last Updated: May 5, 2026

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Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies
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Myo-mechanical Analysis of Isolated Skeletal Muscle
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Main Results:

  • Antisense oligonucleotide delivery via bubble liposomes increased chloride channel (CLCN1) protein expression.
  • Demonstrated amelioration of myotonia in treated myotonic dystrophy mouse models.
  • Identified Manumycin A as a small molecule compound that corrects aberrant Clcn1 splicing in vivo.

Conclusions:

  • Bubble liposome-mediated antisense oligonucleotide delivery is a promising strategy for myotonic dystrophy treatment.
  • Manumycin A represents a potential therapeutic agent for myotonic dystrophy by correcting Clcn1 gene splicing.
  • These findings open new avenues for developing effective treatments for myotonic dystrophy.