Functional ultrasound and brain connectivity reveal central nervous system compromise in Trembler-J mice model of Charcot-Marie-Tooth disease.

Autor: Anzibar Fialho M; Laboratorio deAcústica Ultrasonora, Instituto de Física, Facultad de Ciencias, Universidad de la República, 11400, Montevideo, Uruguay.; Física No Lineal, Instituto de Física de Facultad de Ciencias, Universidad de la República, 11400, Montevideo, Uruguay., Martínez Barreiro M; Laboratorio de Biología Celular del Sistema Nervioso Periférico, Departamento de Proteínas y ÁcidosNucleicos, Instituto de Investigaciones Biológicas Clemente Estable, 11600, Montevideo, Uruguay., Vázquez Alberdi L; Laboratorio de Biología Celular del Sistema Nervioso Periférico, Departamento de Proteínas y ÁcidosNucleicos, Instituto de Investigaciones Biológicas Clemente Estable, 11600, Montevideo, Uruguay., Damián JP; Departamento de Biociencias Veterinarias, Facultad de Veterinaria, Universidad de la República, 13000, Montevideo, Uruguay.; Núcleo de Bienestar Animal, Facultad de Veterinaria, Universidad de la República, 13000, Montevideo, Uruguay., Di Tomaso MV; Departamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, 11600, Montevideo, Uruguay., Baranger J; Institute Physics for Medicine Paris, Inserm U1273, ESPCI Paris, CNRS UMR 8063, PSL University, 75015, Paris, France., Tanter M; Institute Physics for Medicine Paris, Inserm U1273, ESPCI Paris, CNRS UMR 8063, PSL University, 75015, Paris, France., Calero M; Chronic Disease Programme (UFIEC), and CIBERNED, Instituto de Salud Carlos III, 28220, Majadahonda, Madrid, Spain., Negreira C; Laboratorio deAcústica Ultrasonora, Instituto de Física, Facultad de Ciencias, Universidad de la República, 11400, Montevideo, Uruguay., Rubido N; Institute for Complex Systems and Mathematical Biology, University of Aberdeen, King's College, AB24 3UE, Aberdeen, UK., Kun A; Laboratorio de Biología Celular del Sistema Nervioso Periférico, Departamento de Proteínas y ÁcidosNucleicos, Instituto de Investigaciones Biológicas Clemente Estable, 11600, Montevideo, Uruguay. akun@fcien.edu.uy.; Sección Bioquímica, Facultad de Ciencias, Universidad de la República, 11400, Montevideo, Uruguay. akun@fcien.edu.uy., Brum J; Laboratorio deAcústica Ultrasonora, Instituto de Física, Facultad de Ciencias, Universidad de la República, 11400, Montevideo, Uruguay. javier.brum@fcien.edu.uy.
Jazyk: angličtina
Zdroj: Scientific reports [Sci Rep] 2024 Dec 03; Vol. 14 (1), pp. 30073. Date of Electronic Publication: 2024 Dec 03.
DOI: 10.1038/s41598-024-80022-z
Abstrakt: 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.
Competing Interests: Declarations. Competing interests: M.T. is co-founder and shareholder of Iconeous, a company which commercializes ultrasonic neuroimaging scanners. The remaining authors declare no competing interests.
(© 2024. The Author(s).)
Databáze: MEDLINE
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