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Spinal Muscular Atrophy

Spinal muscular atrophy (SMA) is a rare autosomal recessive motor neurodegenerative disease and is the leading genetic cause of death among infants and toddlers. Several mouse models have been developed and scientists at PsychoGenics have contributed to the behavioral characterization of several of them. PsychoGenics has established a large colony of the SMNΔ7 neonatal mouse model of SMA. The SMNΔ7 neonatal model has been well phenotyped and characterized at PsychoGenics (El-Khodor et. al. 2008). By using its in vivo neonatal drug screening platform, PsychoGenics can evaluate new chemical entities, biologics and gene therapies in the SMNΔ7 neonates.

To view phenotypic data, click on any of the experimental tests below:

Body Weight

BodyWeight

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Survival

Survival

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Righting Reflex

The ability of one animal to right itself which is a measure of motor coordination. A pre-righting reflex scores the time (in seconds) that it takes one animal to right itself. The animal is given a maximum of 30 seconds to perform this assessment, and a value of 0-30 is entered (this value is the exact time it takes the animal to right itself). Immediately after, ten (10) consecutive righting reflex trials (3 sec each) are made and the number of successful righting out of 10 trials is recorded. Values presented as mean ± one standard error of the mean (SEM).

Righting Reflex

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Geotaxis

This test evaluates motor coordination and vestibular sensitivity. Animals are placed head facing down on an inclined plane and latency to re-orient head up is recorded.

Geotaxis

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Hindlimb Suspension Test

The tube test is performed in 2 consecutive trials. There is no time limit for either tube test trial. In each trial, the mouse is placed face down, hanging by its hind-limbs in a plastic 50 ml centrifuge tube. The number of pulls to escape the tube is recorded. Values presented as mean ± one standard error of the mean (SEM).

Hindlimb Suspension Test

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  • tel: (914) 406-8019
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