Animal Models of Motor and Sensory Neuron Disease

Motor and sensory neuron diseases are incurable and lethal neurodegenerative diseases, that are mainly caused by the loss of function of the nervous systems over time. Although rare, all forms of motor and sensory neuron diseases are accompanied by severe clinical symptoms. The most common types include amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), progressive bulbar palsy (PBP), progressive muscular atrophy (PMA), Kennedy's disease, and post-polio syndrome.

Pathways of Motor and Sensory

Sensory and motor pathways control the body's movements, including respiration, myocardial contraction, eyelid, and hands movement, etc. Usually, sensory neurons receive information (input) and transmit it to the spinal cord and brain through the ascending axons of sensory neurons. Then, the motor neurons deliver signals to the muscles (output) from the motor cortex to induce muscle contraction. Neuron degeneration of motor and sensory pathways may lead to muscle weakness and loss of tone.

Animal Models of ALS

Animal models are useful tools to address the origin of neuronal dysfunction, the mechanisms of degeneration, and the validation of therapeutic strategies. For the most prominent motor and sensory neuron disease - ALS, scientists have developed a series of animal models to help discover effective drugs and understand the mechanism of how these diverse molecular changes lead to selective death of motor neurons.

Table.1 Animal models of ALS. (Valetdinova, 2015)

Animal Models of SMA

Spinal muscular atrophy is a genetic disorder characterized by degeneration of motor neurons in the anterior horns of the spinal cord that leads to muscle atrophy, palsy, and death. The most common cause is a deletion of the SMN1 gene on chromosome 5 (95%). Based on the manipulations with the SMN gene, many animal models have been developed.

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