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Related Concept Videos

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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Classification of Skeletal Muscle Fibers01:48

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Skeletal muscles continuously produce ATP to provide the energy that enables muscle contractions. Skeletal muscle fibers can be categorized into three types based on differences in their contraction speed and how they produce ATP, as well as physical differences related to these factors. Most human muscles contain all three muscle fiber types, albeit in varying proportions.
Slow-Twitch Muscle Fibers
Slow oxidative, muscle fibers appear red due to large numbers of capillaries and high levels of...
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The Sarcomere01:08

The Sarcomere

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A sarcomere is a microscopic segment repeating in a myofibril. The sarcomere fundamentally consists of two main myofilaments: thick filaments called myosin and thin filaments called actin. These filaments interact by sliding past each other in response to stimulus. In addition to myosin and actin, several other proteins, such as tropomyosin, troponin, titin, nebulin, myomesin, α-actinin, and dystrophin, play crucial roles in regulating, structuring, and functioning of the sarcomere.
Each...
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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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Types of Intermediate Filaments01:31

Types of Intermediate Filaments

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The intermediate filaments are an essential component of the cytoskeleton. Presently six types of intermediate filament have been identified. Type I and II are acidic and basic keratin proteins. Type III is of mesodermal origin and comprises four proteins: vimentin, desmin, glial fibrillary acidic protein (GFAP), and peripherin. Vimentin is commonly found in mesenchymal cells, desmin in muscle cells, GFAP in astrocytes, while peripherin is found in peripheral nervous system neurons (PNS). Type...
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Fibril-associated Collagen01:11

Fibril-associated Collagen

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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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Related Experiment Video

Updated: Jan 3, 2026

Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
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Myofibromatosis.

Kanika Rastogi1, Lavleen Singh2

  • 1Pathology, Chacha Nehru Bal Chikitsalaya, Geeta Colony, Delhi, India.

Fetal and Pediatric Pathology
|November 19, 2019
PubMed
Summary

This study presents a case of childhood myofibromatosis, a rare mesenchymal tumor. Genetic analysis, including SRF-RELA fusions, aids in differentiating subtypes of myofibromas and myopericytomas.

Area of Science:

  • Pediatric Pathology
  • Molecular Genetics
  • Skeletal System Neoplasms

Background:

  • Myofibromatosis is a childhood mesenchymal disorder characterized by specific microscopic features.
  • Genetic mutations like PDGFRB and SRF-RELA fusions are implicated in familial and cellular variants.

Observation:

  • An 11-year-old male presented with an infraorbital mass.
  • Microscopic examination revealed myofibroma with SMA immunopositivity.
  • Differential diagnoses included nodular fasciitis and fibromatosis.

Findings:

  • The case highlights the clinico-pathological features of myofibromatosis.
  • SRF-RELA gene fusions may help differentiate myofibromas/myopericytomas.
  • PDGFRB mutations may distinguish familial myofibromas.
Keywords:
Myofibromatosischildhoodmyofibroblastic

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Implications:

  • Understanding genetic fusions can refine the classification of myofibromatosis.
  • This research contributes to the differential diagnosis and management of pediatric mesenchymal tumors.
  • Further research into genetic markers may lead to targeted therapies.