Functional ultrasound in the brain of TrJ mice, a model of the neurodegenerative disease of Charcot Marie Tooth

Abstract

Charcot-Marie-Tooth’s (CMT) disease is the most commonly human inherited neurological disorder, and is described as a peripheral nervous alteration. Trembler-J (TrJ) mice model this condition with the same spontaneous mutation as in human variant CMT1E . Recent studies in TrJ evidenced alterations in the central nervous system (Damián et al 2021, Biomolecules). To further verify this hypothesis, in this work we used functional ultrasound (fUS) to examine the vascular brain's response to an external stimulus. To our understanding, this is the first neurological functional analysis that studies this disease.