Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the degeneration of motor neurons in the brain and spinal cord, with average life expectancy of 3 to 5 years following diagnosis. Primary clinical manifestations include progressive loss of motor functions — movement, speech, and swallowing — leading to paralysis and ultimately respiratory failure.

50+
Neurological Disease Mouse Models
500+
Studies Completed
10+
IND-Enabling Studies

Advantages

Our Advantages

Targeted Gene Models

ALS models that carry clinically relevant mutations — SOD1 G93A and TDP43 A315T — replicate key features of human ALS, providing predictive platforms for gene therapy and small-molecule screening.

Comprehensive Phenotypingent

Our models enable longitudinal monitoring of motor function, muscle atrophy, body weight, and motor neuron loss, supporting robust efficacy and safety evaluation in preclinical ALS studies.

Flexible Study Design

From model selection to experimental endpoints, our team works closely with sponsors to tailor study protocols that align with specific research goals and regulatory requirements for ALS disease therapies.

Model Library

Case Studies

Case Studies & Validation Data

SOD1 G93A Motor Dysfunction Study

Age-Dependent Motor Dysfunction and Muscle Atrophy of B6-hSOD1 G93A, hSOD1 Mice

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Age-Dependent Motor Dysfunction and Muscle Atrophy of B6-hSOD1 G93A, hSOD1 Mice

Panel A and B: Male mice exhibited motor dysfunction at 5 months old and female mice delayed the onset of motor dysfunction by about 1 months.

All data represented as mean ± SEM. *P<0.05, **P<0.01, ***P<0.001, ****P<0.0001, two-way ANOVA.

Panel C: Representative sections of gastrocnemius muscle from B6-hSOD1 G93A, hSOD1 mice at different ages by H&E staining. The results showed that the mice developed muscle atrophy and inflammatory cell infiltration at 3-5 months of age. Black arrow, inflammatory cells infiltration. Green arrow, muscle fiber atrophy. Scale bar: top panel, 200 μm; bottom panel, 50 μm.

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