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Please use this identifier to cite or link to this item: https://hdl.handle.net/20.500.12008/48667 How to cite
Title: Functional ultrasound and brain connectivity reveal central nervous system compromise in Trembler-J mice model of Charcot-Marie-Tooth disease
Authors: Anzibar Fialho, Maximiliano
Martínez Barreiro, Mariana
Vázquez Alberdi, Lucía
Damián Cabrera, Juan Pablo
Di Tomaso, María Vittoria
Baranger, Jerome
Tanter, Mickael
Calero, Miguel
Negreira, Carlos
Rubido, Nicolás
Kun González, Alejandra E.
Brum, Javier
Type: Artículo
Keywords: Biological physics, Diseases of the nervous system, Imaging techniques, Neuro–vascular interactions, Neuroscience, Peripheral nervous system, Physiology, Somatosensory system
Issue Date: 2024
Abstract: The Charcot-Marie-Tooth-1E (CMT1E) disease is typically described as a peripheral neuropathy in humans, causing decreased nerve conduction, spastic paralysis, and tremor. The Trembler-J (TrJ) mice serve as a high fidelity model of this disease. Here, we use functional ultrasound (fUS) and functional connectivity (FC) to analyze TrJ mice’s brain activity during sensory stimulation and resting state experiments against wild type (WT) mice - the healthy counterpart. fUS is an imaging technique that measures cerebral blood volume (CBV) temporal changes. We study these changes in the primary somatosensory cortex barrel field (S1BF) of both mice populations during periodic vibrissae stimulation, measuring the number of pixels that correlate to the stimulation (i.e., the size of the activation area), the average correlation of these pixels (i.e., the response strength), and the CBV’s rate of change for each stimulation (i.e., the hemodynamic response). Then, we construct a FC matrix for each genotype and experiment by correlating the CBV signals from the eight cortical regions defined by the Paxinos and Franklin atlas. Our results show that TrJ mice have significantly diminished neurovascular responses and altered brain connectivity with respect to WT mice, pointing to central nervous system effects that could shift our understanding of the CMT1E disease.
Publisher: Nature
IN: Scientific Reports, 2024, 14: 30073
Sponsors: ANII: FCE_1_2019_1_155539
ANII: POS_NAC_M_2020_1_164127
ANII: POS_FCE_2020_1_1009181
ANII: POS_NAC_2022_1_173578
CSIC: 2018-FID 13-Grupo ID 722.
CSIC: I+D 497725-2023-2027
Citation: Anzibar Fialho, M, Martínez Barreiro, M, Vázquez Alberdi, L [y otros autores]. "Functional ultrasound and brain connectivity reveal central nervous system compromise in Trembler-J mice model of Charcot-Marie-Tooth disease". Scientific Reports. [en línea] 2024, 14: 30073. 11 h. DOI: 10.1038/s41598-024-80022-z
License: Licencia Creative Commons Atribución (CC - By 4.0)
Appears in Collections:Publicaciones académicas y científicas - Facultad de Ciencias

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