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Related Concept Videos

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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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
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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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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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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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Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
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Related Experiment Video

Updated: Mar 26, 2026

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
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[Amyotrophic lateral sclerosis, a heterogeneous disorder].

Michael A van Es1, Esther T Kruitwagen-van Reenen, Carin D Schröder

  • 1Universitair Medisch Centrum Utrecht, Utrecht.

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|February 11, 2016
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Summary

Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease with genetic links. Gene-targeted therapies, like antisense oligonucleotides, offer promising new treatment avenues for ALS patients.

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Area of Science:

  • Neurology
  • Genetics
  • Disease Pathogenesis

Background:

  • Amyotrophic lateral sclerosis (ALS) involves progressive motor neuron loss, causing weakness and spasticity.
  • Diagnosis is often by exclusion, and ALS shares a spectrum with frontotemporal dementia (FTD).
  • ALS is genetically heterogeneous, with over 20 implicated genes, and 5-10% of cases are familial.

Purpose of the Study:

  • To summarize the current understanding of ALS pathogenesis and genetic factors.
  • To highlight the symptomatic nature of current ALS treatments and the focus on quality of life.
  • To discuss emerging gene-targeted therapies for ALS.

Main Methods:

  • Review of genetic factors contributing to ALS.
  • Analysis of the relationship between ALS and FTD.
  • Evaluation of current and novel therapeutic strategies.

Main Results:

  • ALS is characterized by motor neuron degeneration and has significant genetic underpinnings.
  • A spectrum exists between ALS and FTD, with overlapping clinical signs.
  • Antisense oligonucleotide strategies represent a promising gene-targeted treatment approach.

Conclusions:

  • Understanding ALS genetics is crucial for developing effective treatments.
  • Current ALS management focuses on symptom control and quality of life.
  • Gene-targeted therapies hold significant promise for future ALS treatment.