Amyotrophic Lateral Sclerosis (ALS) is a late onset progressive paralytic disorder marked by the degeneration of motor neurons in the brain and spinal cord. ALS patients are classified into two main categories, sporadic (SALS) and familial (FALS). A rather large subset (~20%) of FALS cases have been linked to mutations in the Cu/Zn superoxide dismutase 1 gene (SOD1)
Transgenic mice expressing the mutant form of human SOD1 become paralyzed as a result of neuronal loss from the brain and spinal cord and die at 5-6 months of age. These mutant mice show a phenotype typical of motor neuron loss which includes severe muscle wasting and hindlimb paralysis. Recent evidence suggests that the appearance of symptoms is associated with an accumulation of detergent-insoluble, sedimentable, forms of mutant SOD1 in spinal cords and brainstems.
The availability of this mutant mouse model has advanced our understanding of the mechanisms involved in ALS, while at the same time supported the development of novel drug treatments in this disease category. PGI offers a large panel of tests using the most widely chosen mouse model of ALS. These tests include standard motor function measures such as grip strength and rotarod as well as more advances computer vision metrics such as NeuroCube® technology for gait measures and SmartCube® technology for whole animal behavior.
To view phenotypic data, click on any of the experimental tests below:
The body weight of the mice is recorded on at regular intervals throughout the duration of the study.
Grip strength is used to assess muscular strength in limb muscles. The animal is lowered toward the platform and gently pulled backwards with consistent force by the experimenter until it releases its grip. The grip force is recorded on the strain gauge.
Motor coordination and exercise capacity are assessed by rotarod. Tests are performed on separate days, with multiple trials per day. Mice are placed on the rotarod and the speed is gradually and uniformly increased over a maximum period of time. The time that each mouse remains on the rotating rod before falling is recorded.
Survival of the mice is recorded and can be cumulatively tracked as the population in the various treatment groups decline.