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

Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

1.9K
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...
1.9K
Nonsense-mediated mRNA Decay02:27

Nonsense-mediated mRNA Decay

10.5K
The Upf proteins that carry out nonsense-mediated decay (NMD) are found in all eukaryotic organisms, including humans. Each protein has an individual role, but they need to work in collaboration. Upf1 is an ATP-dependent RNA helicase that unwinds the RNA helix. Because Upf1 can unwind any RNA, Upf2 and Upf3 are required to help Upf1 discriminate between nonsense and normal mRNAs.
Usually, Upf3 binds to an Exon Junction Complex (EJC) at mRNA splice sites. If a ribosome fully translates the mRNA,...
10.5K
Mutations01:39

Mutations

80.0K
Overview
80.0K
Abnormal Proliferation02:23

Abnormal Proliferation

4.4K
Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the...
4.4K

You might also read

Related Articles

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

Sort by
Same author

Structure and potential role of T6SS effector PdpC in Francisella tularensis intracellular lifestyle.

Communications biology·2026
Same author

The tumour suppressor RBM5 activates the helicase DHX15 to regulate splicing.

Research square·2026
Same author

The tumour suppressor RBM5 activates the helicase DHX15 to regulate splicing.

bioRxiv : the preprint server for biology·2026
Same author

Discovery and cryoEM structure of FPM13, a periplasmic metalloprotein unique to Francisella.

PLoS pathogens·2026
Same author

Cryo-EM structures reveal a conserved architecture for raiA noncoding RNA.

Nucleic acids research·2026
Same author

Bridging structure and function: artificial intelligence-based modelling of kidney proteins.

Nature reviews. Nephrology·2026

Related Experiment Video

Updated: Jun 5, 2025

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
09:39

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Published on: July 29, 2016

15.3K

Native DGC structure rationalizes muscular dystrophy-causing mutations.

Shiheng Liu1,2, Tiantian Su1,2, Xian Xia1,2

  • 1Department of Microbiology, Immunology and Molecular Genetics, University of California, Los Angeles, Los Angeles, CA, USA.

Nature
|December 11, 2024
PubMed
Summary
This summary is machine-generated.

Duchenne muscular dystrophy (DMD) is caused by defects in the dystrophin-glycoprotein complex (DGC). This study reveals the DGC

More Related Videos

In Vivo Modeling of the Morbid Human Genome using Danio rerio
12:31

In Vivo Modeling of the Morbid Human Genome using Danio rerio

Published on: August 24, 2013

20.6K
Why Quantification Matters: Characterization of Phenotypes at the Drosophila Larval Neuromuscular Junction
10:41

Why Quantification Matters: Characterization of Phenotypes at the Drosophila Larval Neuromuscular Junction

Published on: May 12, 2016

8.1K

Related Experiment Videos

Last Updated: Jun 5, 2025

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
09:39

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

Published on: July 29, 2016

15.3K
In Vivo Modeling of the Morbid Human Genome using Danio rerio
12:31

In Vivo Modeling of the Morbid Human Genome using Danio rerio

Published on: August 24, 2013

20.6K
Why Quantification Matters: Characterization of Phenotypes at the Drosophila Larval Neuromuscular Junction
10:41

Why Quantification Matters: Characterization of Phenotypes at the Drosophila Larval Neuromuscular Junction

Published on: May 12, 2016

8.1K

Area of Science:

  • Molecular biology
  • Structural biology
  • Genetics

Background:

  • Duchenne muscular dystrophy (DMD) is a severe genetic disorder.
  • The dystrophin-glycoprotein complex (DGC) is crucial for muscle integrity.
  • The molecular structure of the DGC was previously unknown.

Purpose of the Study:

  • To determine the native structure of the DGC.
  • To elucidate the molecular architecture and interactions within the DGC.
  • To understand how mutations in DGC components cause muscular dystrophy.

Main Methods:

  • Cryo-electron microscopy of rabbit DGC.
  • Biochemical analyses of DGC components and interactions.

Main Results:

  • The study determined the native cryo-electron microscopy structure of the rabbit DGC.
  • An extracellular β-helix formed by sarcoglycans provides a platform for matrix interaction.
  • Specific domains of dystrophin interact with other DGC components and link to intracellular actin.

Conclusions:

  • The DGC structure reveals how it links the extracellular matrix to the cytoskeleton.
  • Findings rationalize over 110 disease-causing mutations in muscular dystrophy subtypes.
  • This structural insight aids in developing therapeutic strategies for DMD.