Amyotrophic Lateral Sclerosis
Validated ALS Models and Research Services for Drug Development
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease or Lou Gehrig’s disease, is a progressive neurodegenerative disease that affects motor neurons responsible for controlling voluntary movement, resulting in muscle weakness, paralysis, and eventually respiratory failure. New drug development is crucial as there are few therapeutics available to halt, delay or reverse the progression of ALS. Partner with Inotiv for the preclinical development for your novel therapeutic for ALS.
SOD1G93A Rat Model of ALS
Mutations in the superoxide dismutase 1 (SOD1) gene are linked to familial ALS. These mutations cause the enzyme to misfold, leading to toxic effects in motor neurons, which results in muscle weakness and paralysis. SOD1G93A transgenic rats from Taconic Biosciences express the G93A mutant form of human SOD1 in the spinal cord, brain, and peripheral tissues. These rats typically display ALS-related symptoms at about 174-207 days of age.
Validation Data
Motor coordination was assessed in SOD1G93A rats (orange line) and wild type controls (red line). SOD1G93A rats exhibited significantly more foot faults than wild type controls during the beam walk test (A) and fell off of the rotarod at lower revolutions per minute (RPM) than wild type controls (B).
Quantification of plasma neurofilament light chain, a clinical biomarker of neurodegeneration, in the plasma of SOD1G93A rats (orange line) and wild type controls (red line) . Circulating neurofilament light chain was detected in the plasma of SOD1G93A rats but not in the plasma from wild type control animals.
Histopathological Services for ALS Models
Histopathological analysis of neural tissue from ALS models can provide insights into the progression of ALS at the microscopic level, identifying specific targets for therapy, and evaluating the effectiveness of new drugs in preventing or reversing cellular damage.
Choline acetyltransferase (ChAT) was detected in sections of lumbar spinal cord (brown staining) from SOD1G93A (A) and wild type controls (B). ChAT, a marker for motor neurons, is a critical enzyme in acetylcholine production, the neurotransmitter responsible for muscle activation.
Learn more about our range of specialty histology and image analysis services.
Additional Endpoints
We also have a broad range of GLP and non-GLP in vivo and in vitro assays that can be customized to provide solutions for your ALS drug discovery program.
Drug Metabolism and Pharmacokinetics (DMPK)
Histology
Pathology
In Vivo Services
Bioanalysis
