Amyotrophic Lateral Sclerosis – SOD1-G93A Transgenic ALS Mouse ModelÂ
Develop novel targeted Amyotrophic Lateral Sclerosis (ALS) therapies by modeling early-stage symptomatic ALS using the SOD1-G93A Transgenic ALS Mouse Model
SOD1-G93A Transgenic ALS Mouse ModelÂ
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.Â
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.Â
✓ Early-onset of motor function impairment.
✓ Behavioural changes can be detected already at young ages in SOD1-G93A transgenic ALS mice and are progressive, making this model a valuable tool for efficacy testing of novel compounds.Â
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.Â
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).Â
Your ALS Research Starts Here.
View study timelines, recommended readouts, and example data featuring behavioral across different ALS mouse models.
SOD1-G93A Transgenic ALS Mouse Model Sample Data
SOD1-G93A Transgenic ALS mice show reduced amplitude in nerve conduction velocity.
Representative schematic and image of experimental set-up. Nerve conduction study is an electrophysiological recording method that measures the speed and the amplitude of an electrical impulse moving through the sciatic nerve of the mouse. Electrophysiological recordings are performed on the sciatic nerve, the largest nerve of the peripheral nervous system, supplying the mouse’s hind limb with both motoric and sensory fiber tracts, using needle electrodes. SODG93A mice (C57bl/6J background, JAX # 004435) show reduced neuromuscular function. Reduction of compound muscle action potential (CMAP) amplitude in SOD1G93A mice at 11 weeks of age (before the onset of motor function impairments).
Neurofilament light chain (NfL) levels in the plasma of SODG93A mice (C57bl/6J background, JAX # 004435).
Compared to WT littermates, SOD1G93A mice show significantly higher concentrations of plasma NfL at 11 weeks of age (before onset of motor function impairments). Increasing NfL in blood (and CSF) is a marker of neuronal injury, confirming the extensive neurodegenerative phenotype observed in ALS mice.Â
SOD1-G93A Transgenic ALS mouse model mice show progressive behavioural changes in the automatic home cages (PhenoTyperâ„¢) 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.
SOD1-G93A Transgenic ALS Mouse Model Readouts
Biological Readouts
Test the efficacy of your treatments with the following biological readouts:Â
 Â
- MSD (e.g. cytokines)Â
- Immunohistochemistry (e.g. SOD1 aggregates, neuromuscular junction)Â
- Immunofluorescence and FISH
The People Behind Your Research
Thomas Vogels, PhD, In Vivo Neurology Study Director
Leads an expert team of scientists with vast experience in our Neurology models to help you choose the right model and guide your optimal study design. We provide the solution to accelerating your drug development.
Discover Other Relevant Neurological Disease Models
Discover InnoSer’s Latest Research
![InnoSer Presents New Data on Tau[P301S] and α-Synuclein Combination Models for Neurodegenerative Research at ADPD 2025](https://www.innoserlaboratories.com/wp-content/uploads/2025/03/ADPD-2025-InnoSer-Neurodegenerative-Disease-Models-1080x628.png)
InnoSer Presents New Data on Tau[P301S] and α-Synuclein Combination Models for Neurodegenerative Research at ADPD 2025
We are excited to announce that InnoSer, as an innovative European preclinical neurodegenerative disease CRO, will be actively participating in the upcoming ADPD 2025 conference, taking place in Vienna, Austria from 1st to 5th April 2025. Â The ADPD 2025 Conference is...
InnoSer Strengthens Neurology Platform with Acquisition of remynd’s CRO Business
InnoSer’s acquisition of remynd’s CRO business further strengthens its neurology platform by integrating specialized neurodegenerative mouse models, expanding its service portfolio for Alzheimer’s and Parkinson’s diseases. Diepenbeek, Belgium – March 4th – InnoSer, a...
Gene Therapy Delivery Strategies: Helping You Address Challenges for CNS Diseases
With several recent gene therapy approvals in the CNS space and more on the horizon, the field of both genetic and neurodegenerative diseases is experiencing a transformative era. However, translating these therapies from preclinical research to clinical application...
How to Advance your Preclinical Research Using Mouse Data to Establish PK/PD Relationships Â
The major objective of early drug development is to select the most promising compounds and establish safe and effective doses and dosing regimens. To do so, an analysis of both the pharmacokinetic (PK) and pharmacodynamic (PD) properties of your novel compound needs...
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.
info@innoserlaboratories.com