SOD1-G93A Transgenic ALS Mouse Model
Develop novel targeted Amyotrophic lateral Sclerosis (ALS) therapies by modelling early-stage symptomatic ALS using the SOD1-G93A Transgenic ALS Mouse Model
Mutations in the superoxide dismutase 1 (SOD1) gene are associated with familial amyotrophic lateral sclerosis (ALS). SOD1 mutations are thought to result in increased oxidative stress and protein aggregation, ultimately leading to a decline in motor function. Several ALS mouse models mimic the progressive loss of motor neurons in the motor cortex, brain stem, and spinal cord. The SOD1-G93A transgenic ALS mouse model express mutant form of SOD1, representing a translationally relevant model for efficacy studies for ALS.
In line, SOD1 mice show significant SOD1 aggregaiton, oxidative stress, motor neuron loss and significant motor impairments. This mouse model of ALS shows mild behavioral phenotypes up until 12 weeks of age, with progressive neuromuscular deficits leading to paralysis and mortality/human endpoint after 20 weeks of age.
As part of InnoSer’s neurology ALS mouse model portfolio, we also offer efficacy studies in mouse models with TDP-43 pathology, including transgenic TDP-43 mouse models as well as seeding TDP-43 mouse models using recombinant and/or patient-derived seeds. However, as each model is unique, modelling distinct pathophysiological aspects of ALS, we recommend you discuss the most appropriate model with our neurology study directors.
InnoSer’s neurology expert team possesses relevant experience in working with multiple therapy types ranging from small molecules, peptides, enzymes, oligonucleotides, gene therapy (viral vectors – e.g.. AAVs) and immunotherapies (antibody/vaccine immunotherapies).
Key characteristics of the SOD1-G93A transgenic ALS mouse model:
- Early-onset of motor function impairment.
- Behavioural changes can be detected already at young ages in SOD1 mice and are progressive, making this model a valuable tool for efficacy testing of novel compounds.
Find the right model for you.
Compare our model capabilities and discover which of our neurology platforms suits your research needs
Key readouts in the SOD1-G93A transgenic ALS mouse model
Test the efficacy of your treatments in the following battery of behavioural tests:
Test the efficacy of your treatments with the following biological readouts:
- MSD (e.g. cytokines)
- Immunohistochemistry (e.g. SOD1 aggregates, neuromuscular junction)
- Immunofluorescence and FISH
Example data featuring the SOD1-G93A mouse model
SOD1-G93A Transgenic ALS mouse model mice show progressive behavioural changes in the automatic home cages (PhenoTyper™) including a reduced frequency to climb on top of their shelter
SOD1-G93A Transgenic ALS Mouse Model show decrease in motor performance assessed by rotarod
The Rotarod is the golden standard of assessing motor performance and learning in mice. The mice are placed on a rotating rod, with increasing rotating speed. Motor performance is measured by the maximal RPM (rounds per minute) at which mice can keep up with the rotating rod. Motor learning can be assessed by training mice on the rod for several trials.
Related ALS mouse models
Neurology Platform Overview
TDP-43 Transgenic Mouse Model
InnoSer offers services with several different transgenic TDP-43 mouse models that replicate the TDP-43 proteinopathy in ALS and FTD patients.
TDP–43 Seeding Mouse Models
InnoSer offers extensive preclinical research experience with TDP-43 seeds using patient-derived brain extracts.
AAALAC Accreditation
InnoSer has earned the AAALAC accreditation, demonstrating our commitment to responsible animal care and use. AAALAC International is a nonprofit organization that promotes the humane treatment of animals in science through voluntary accreditation and assessment programs. Our accreditation is valid for three years, incl. 2023. Read more about the AAALAC accreditation programme here.
Animal Welfare
The 3Rs impact everything from policy and regulatory change to the development and uptake of new technologies and approaches. This is why Innoser has ongoing commitment and monitoring of these processes. The steps we practice maximize our ability to replace, reduce and refine animal involvement and facilitate our commitment to these principles when it comes to research and drug development.
Need more information?
If you have any questions about how we can help accelerate your research,
then let us know