The goal of the research was to study the pathology of myelin and axon in PMP22 overexpressing mice, a mouse model often used for CMT1A. The research question that is asked in the research is what is the change to the mice’s muscle tone, electrophysiology, and histology in the course of 1.5 years?
The experiment showed that the phenotype that is measured through noninvasive measures is indicative of certain morphology of the cells in mice and that the disease gets worse both in behavior and in morphology, histology, and pathology as the mice age. In addition, the C3 mice, in general, had less severe symptoms compared to the C22 mice but had symptoms whereas the control did not have any symptoms. In general, the C3 mice would be the better model for CMT1A.
The impact of this research on the disease is the creation of new model of CMT1A mice, which have a relatively more mild symptoms, and as a result would be more effective model compared to C22 model. This is important because the severity of C22 mice means that the treatment that work in C22 model would less likely be able to work in humans because humans generally do not have symptoms that severe. By having a less severe model, it will allow better replication in humans, saving time and money.
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