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Characterizing The Onset And Progression Of Charcot-Marie-Tooth Neuropathy In H304r Mutant Mice, Aaron Ledray
Characterizing The Onset And Progression Of Charcot-Marie-Tooth Neuropathy In H304r Mutant Mice, Aaron Ledray
HIM 1990-2015
Dynein is a motor protein complex that transports various types of intracellular cargos from the cell periphery towards the cell center. Dynein mutations are linked to several neurodegenerative diseases, including Charcot-Marie-Tooth disease (CMT). A mouse model of CMT was generated with a knock-in H304R dynein allele. This mutation at position 304 corresponds to the H306R mutation found in humans that can cause CMT. Here, a behavioral test was developed to study the onset and progression of CMT symptoms in these mice. In the tail suspension test, mice were suspended briefly by their tails and the posture of their hind limbs …