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

You might also read

Related Articles

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

Sort by
Same author

TRPV1 Agonist Capsaicin Enhances Oxidative-Stress Resistance and Regeneration in Dorsal Root Ganglia and Schwann Cells.

Cells·2026
Same author

Emergent cytotoxicity and mitochondrial alterations induced by a heterobimetallic Re(I)/Au(I) complex.

Chemical communications (Cambridge, England)·2026
Same author

Patient-derived glioblastoma cultures preserve respiration phenotypes during ex vivo maintenance and show sex-associated differences in migration.

Acta neuropathologica communications·2026
Same author

Molecular and clinical characteristics of patients with non-small cell lung cancer (NSCLC) harboring KRAS Q61 mutations to assess therapeutic responses.

Clinical cancer research : an official journal of the American Association for Cancer Research·2026
Same author

Global disparities in access to diagnostics and therapies for advanced non-small cell lung cancer: from discovery to delivery - a review.

Frontiers in oncology·2026
Same author

A Novel 3D Semi-Automated Full Quantification Technique for Detection of Intraneural Phospho-α-Synuclein in Skin Biopsies.

European journal of neurology·2026

Related Experiment Video

Updated: Aug 28, 2025

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS
12:35

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS

Published on: March 17, 2012

28.2K

ROS scavengers decrease γH2ax spots in motor neuronal nuclei of ALS model mice in vitro.

Maya Junghans1, Felix John1, Hilal Cihankaya1

  • 1Department of Cytology, Institute of Anatomy, Ruhr University Bochum, Bochum, Germany.

Frontiers in Cellular Neuroscience
|September 19, 2022
PubMed
Summary

Oxidative stress and impaired reactive oxygen species (ROS) detoxification contribute to DNA damage in motor neurons of Wobbler mice, a model for Amyotrophic Lateral Sclerosis (ALS). ROS scavengers reduced DNA damage, suggesting redox homeostasis is key for ALS therapy.

Keywords:
Mito-TEMPON-Acetyl-L-CysteineWobblerdouble strand breaksglutathione ethyl esterneuroprotectionp53bp1

More Related Videos

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
06:49

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS

Published on: October 6, 2015

19.9K
Treating SCA1 Mice with Water-Soluble Compounds to Non-Specifically Boost Mitochondrial Function
11:47

Treating SCA1 Mice with Water-Soluble Compounds to Non-Specifically Boost Mitochondrial Function

Published on: January 22, 2017

10.7K

Related Experiment Videos

Last Updated: Aug 28, 2025

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS
12:35

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS

Published on: March 17, 2012

28.2K
A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
06:49

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS

Published on: October 6, 2015

19.9K
Treating SCA1 Mice with Water-Soluble Compounds to Non-Specifically Boost Mitochondrial Function
11:47

Treating SCA1 Mice with Water-Soluble Compounds to Non-Specifically Boost Mitochondrial Function

Published on: January 22, 2017

10.7K

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by motor neuron loss.
  • Oxidative stress is implicated in ALS, but specific mechanisms within motor neurons remain unclear.
  • Investigating reactive oxygen species (ROS) and DNA damage in a Wobbler mouse model is crucial.

Purpose of the Study:

  • To examine ROS detoxification pathways in motor neurons of the Wobbler mouse model.
  • To determine the link between ROS and DNA double-strand breaks (DSBs) in ALS.
  • To evaluate the therapeutic potential of ROS scavengers in vitro.

Main Methods:

  • Live cell imaging (Dihydroethidium) to quantify ROS production in motor neurons.
  • Quantitative PCR (qPCR) and Western blotting to assess antioxidant molecule expression.
  • Immunofluorescence staining and qPCR to measure DNA damage response proteins (p53bp1, H2ax); in vitro ROS scavenger experiments.

Main Results:

  • Elevated ROS levels and increased DNA damage markers (p53bp1, γH2ax) in Wobbler mouse motor neurons.
  • Altered expression of antioxidant molecules indicates impaired ROS detoxification.
  • In vitro ROS scavengers reduced γH2ax foci, mitigating ROS-induced DNA damage.

Conclusions:

  • Redox homeostasis is critical for motor neuron survival in ALS.
  • Deficits in ROS detoxification contribute to neurodegeneration in ALS.
  • Multimodal therapeutic strategies targeting multiple pathways are necessary for effective ALS treatment.