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Disorders of the Skeletal Muscle01:28

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
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Three main types of fibers are secreted by fibroblasts: collagen fibers, elastic fibers, and reticular fibers. Collagen fiber is made from fibrous protein subunits linked together to form a long, straight fiber. Collagen fibers, while flexible, have great tensile strength, resist stretching, and give ligaments and tendons their characteristic resilience and strength. These fibers hold connective tissues together, even during the body's movement.
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Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
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Structural proteins are a category of proteins responsible for functions ranging from cell shape and movement to providing support to major structures such as bones, cartilage, hair, and muscles. This group includes proteins such as collagen, actin, myosin, and keratin.
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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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Transmembrane Collagens in Neuromuscular Development and Disorders.

Tomoko Wakabayashi1

  • 1Department of Innovative Dementia Prevention, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

Frontiers in Molecular Neuroscience
|February 4, 2021
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Summary

Transmembrane collagens, like Collagen XXV and Collagen XIII, are crucial for neuromuscular development and function. Disruptions in these proteins can lead to serious motor disorders and neuromuscular junction defects.

Keywords:
axon guidancecollagenextracellular matrixmotor neuronmyasthenia gravisneuromuscular junction (NMJ)

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Area of Science:

  • Cell Biology
  • Neuroscience
  • Developmental Biology

Background:

  • Neuromuscular development relies on complex molecular interactions for motor axon targeting.
  • Transmembrane collagens, particularly Membrane-Associated Collagens with Interrupted Triple Helices (MACITs), are increasingly recognized for their roles in neuromuscular formation.
  • Collagen XXV and Collagen XIII are key MACITs involved in distinct aspects of neuromuscular development and function.

Purpose of the Study:

  • To review recent advances in understanding the roles of transmembrane collagens in neuromuscular development.
  • To explore the molecular mechanisms underlying the function of transmembrane collagens in the neuromuscular system.
  • To highlight the implications of transmembrane collagen dysfunction in human neuromuscular disorders.

Main Methods:

  • Literature review of studies on transmembrane collagens in neuromuscular development.
  • Analysis of genetic and functional data from various model organisms, including humans and *C. elegans*.
  • Synthesis of current knowledge on the roles of Collagen XXV, Collagen XIII, COL-99, and UNC-122.

Main Results:

  • Collagen XXV is essential for motor axon growth during muscle development, and its mutations cause ocular motor disorders.
  • Collagen XIII is vital for the formation and maintenance of neuromuscular junctions (NMJs), with dysfunction leading to congenital myasthenic syndrome.
  • Transmembrane collagens exhibit conserved functions across species, with *C. elegans* models like COL-99 and UNC-122 demonstrating roles in motor innervation and NMJ integrity.

Conclusions:

  • Transmembrane collagens are critical regulators of neuromuscular development, axon guidance, and neuromuscular junction stability.
  • Understanding the molecular mechanisms of transmembrane collagens offers insights into the pathogenesis of congenital neuromuscular disorders.
  • Further research into transmembrane collagens holds potential for therapeutic strategies targeting neuromuscular diseases.