Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Lysosomal Hydrolases01:22

Lysosomal Hydrolases

4.0K
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,...
4.0K
The Extracellular Matrix01:42

The Extracellular Matrix

85.6K
Overview
85.6K
Microscopic Anatomy of Skeletal Muscles01:13

Microscopic Anatomy of Skeletal Muscles

18.1K
Skeletal muscle cells, also called muscle fibers, are distinctly elongated, multi-nucleated, slender biological units. They are packed with specialized structures designed to facilitate their primary function, which is contraction.
The muscle sarcolemma is a plasma membrane enclosing each muscle cell that conducts electrical signals called action potentials. The sarcolemma extends into the cell to form T-tubules, ensuring the neural impulses are uniformly distributed across the entire muscle...
18.1K
Extracellular Matrix01:26

Extracellular Matrix

3.9K
Unlike epithelial tissue, which is composed of cells closely packed with little or no extracellular space in between, connective tissue cells are dispersed in a matrix. This extracellular matrix (ECM) is composed of fibrous proteins like collagen, elastin, and fibronectin in a ground substance consisting of interstitial fluid, cell adhesion proteins, and proteoglycans. The proteoglycans form a gel-like material in the spaces between cells and provide hydration, buffering, binding, and force...
3.9K
Overview of Skeletal Muscle01:15

Overview of Skeletal Muscle

13.2K
Skeletal muscles are composed of a bundle of muscle fibers and are attached to bones through tendons. Each skeletal muscle fiber is a single muscle cell. The sarcolemma, the plasma membrane of a skeletal muscle cell, consists of a lipid bilayer and glycocalyx that supports muscle fibers. The sarcolemma extends into the muscle cells to form tubular structures called transverse or T-tubules. Each side of the T-tubules consists of a membrane-bound structure called the sarcoplasmic reticulum,...
13.2K
Lysosomes01:31

Lysosomes

20.7K
Lysosomes are membrane-enclosed spherical sacs derived from the Golgi apparatus. The most important function of the lysosome is degrading macromolecules and biological polymers that are released during membrane trafficking events such as the secretory, endocytic, autophagic, and phagocytic pathways. The degradation is carried out by several hydrolytic enzymes active in an acidic environment of the lysosomal lumen. These acid hydrolases are involved in cellular processes such as cell signaling,...
20.7K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Interactions Between Feeding Protocols, Larval Density, and Methylene Blue During Zebrafish Larval Development.

Zebrafish·2026
Same author

Epigenetic small molecule screening identifies a new HDACi compound for ameliorating Duchenne muscular dystrophy.

bioRxiv : the preprint server for biology·2025
Same author

Standardization of zebrafish drug testing parameters for muscle diseases.

Disease models & mechanisms·2024
Same author

Super-Resolution Imaging Reveals the Nanoscale Distributions of Dystroglycan and Integrin Itga7 in Zebrafish Muscle Fibers.

Biomedicines·2023
Same author

Beneficial impacts of neuromuscular electrical stimulation on muscle structure and function in the zebrafish model of Duchenne muscular dystrophy.

eLife·2022
Same author

NAD+ improves neuromuscular development in a zebrafish model of FKRP-associated dystroglycanopathy.

Skeletal muscle·2019
Same journal

An Overview of How Epigenetics, MicroRNA-21, and Endocrine Disrupting Compounds Affect Oocyte Maturation and Pre-Implantation Embryo Development.

Journal of developmental biology·2026
Same journal

The Gut Microbiome in Early Ontogeny: Implications for Brain and Immune System Development.

Journal of developmental biology·2026
Same journal

Gestational Dynamics of Hofbauer Cells in the Human Placenta: Distribution, Morphology, and Immunophenotype.

Journal of developmental biology·2026
Same journal

Spatiotemporal Profiling Defines the Epithelial and Mesenchymal Transition Window in Embryonic Lung Morphogenesis.

Journal of developmental biology·2026
Same journal

The Association Between the Progressive Motility of Bovine Spermatozoa and the Developmental Morphokinetics of In Vitro-Derived Embryos.

Journal of developmental biology·2026
Same journal

Expression and Role of Colony Stimulating Factor 1 Receptor During Odontogenesis.

Journal of developmental biology·2026
See all related articles

Related Experiment Video

Updated: Oct 11, 2025

Enzymatic Isolation of Skeletal Muscle Interstitial Extracellular Vesicles
08:50

Enzymatic Isolation of Skeletal Muscle Interstitial Extracellular Vesicles

Published on: February 7, 2025

1.2K

Lysosomal Function Impacts the Skeletal Muscle Extracellular Matrix.

Elizabeth C Coffey1, Mary Astumian2, Sarah S Alrowaished1

  • 1School of Biology and Ecology, University of Maine, Orono, ME 04469, USA.

Journal of Developmental Biology
|November 29, 2021
PubMed
Summary
This summary is machine-generated.

Dysregulated lysosomal pH disrupts muscle homeostasis by affecting extracellular matrix proteins at the myotendinous junction. This impacts muscle degeneration in Sphingolipid transporter 1 (spns1) mutants.

Keywords:
basement membranelysosomal myopathymyotendinous junctionskeletal musclespinsterzebrafish

More Related Videos

In Situ Immunofluorescent Staining of Autophagy in Muscle Stem Cells
08:35

In Situ Immunofluorescent Staining of Autophagy in Muscle Stem Cells

Published on: June 12, 2017

10.3K
Preparation of 3D Decellularized Matrices from Fetal Mouse Skeletal Muscle for Cell Culture
07:44

Preparation of 3D Decellularized Matrices from Fetal Mouse Skeletal Muscle for Cell Culture

Published on: March 3, 2023

1.5K

Related Experiment Videos

Last Updated: Oct 11, 2025

Enzymatic Isolation of Skeletal Muscle Interstitial Extracellular Vesicles
08:50

Enzymatic Isolation of Skeletal Muscle Interstitial Extracellular Vesicles

Published on: February 7, 2025

1.2K
In Situ Immunofluorescent Staining of Autophagy in Muscle Stem Cells
08:35

In Situ Immunofluorescent Staining of Autophagy in Muscle Stem Cells

Published on: June 12, 2017

10.3K
Preparation of 3D Decellularized Matrices from Fetal Mouse Skeletal Muscle for Cell Culture
07:44

Preparation of 3D Decellularized Matrices from Fetal Mouse Skeletal Muscle for Cell Culture

Published on: March 3, 2023

1.5K

Area of Science:

  • Muscle physiology
  • Cellular biology
  • Biochemistry

Background:

  • Muscle development and homeostasis rely on protein turnover, regulated by lysosomal pH.
  • Sphingolipid transporter 1 (spns1) maintains lysosomal pH; spns1 mutants exhibit premature senescence.
  • The effect of disrupted lysosomal pH on muscle development and homeostasis is unclear.

Purpose of the Study:

  • Investigate the impact of dysregulated lysosomal pH on muscle development and homeostasis.
  • Identify molecular mechanisms underlying muscle degeneration in spns1 mutants.

Main Methods:

  • Analysis of muscle development and degeneration in spns1 mutants.
  • Assessment of extracellular matrix (ECM) protein expression at the myotendinous junction (MTJ).
  • Investigated the roles of laminin 111 and MMP-9 in muscle degeneration.

Main Results:

  • Muscle development was normal in spns1 mutants before degeneration onset.
  • Dysregulation of ECM at the MTJ correlated with muscle degeneration.
  • Laminin 111 upregulation mitigated degeneration; MMP-9 upregulation, induced by tnfsf12 signaling, did not.

Conclusions:

  • Dysregulated lysosomal pH affects ECM protein expression at the MTJ.
  • Laminin 111 plays a protective role in muscle degeneration.
  • Lysosomal pH is critical for maintaining muscle homeostasis through ECM regulation.