In Vivo Ultrafast Doppler Imaging Combined with Confocal Microscopy and Behavioral Approaches to Gain Insight into the Central Expression of Peripheral Neuropathy in Trembler-J Mice

The main human hereditary peripheral neuropathy (Charcot-Marie-Tooth, CMT), manifests in progressive sensory and motor deficits. Mutations in the compact myelin protein gene pmp22 cause more than 50% of all CMTs. CMT1E is a subtype of CMT1 myelinopathy carrying micro-mutations in pmp22. The Trembler...

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Published in:	Biology (Basel, Switzerland) Vol. 12; no. 10; p. 1324
Main Authors:	Martínez Barreiro, Mariana, Vázquez Alberdi, Lucia, De León, Lucila, Avellanal, Guadalupe, Duarte, Andrea, Anzibar Fialho, Maximiliano, Baranger, Jérôme, Calero, Miguel, Rubido, Nicolás, Tanter, Mickael, Negreira, Carlos, Brum, Javier, Damián, Juan Pablo, Kun, Alejandra
Format:	Journal Article
Language:	English
Published:	Basel MDPI AG 01-10-2023 MDPI
Subjects:	Anxiety behavioral tests Blood Blood flow Brain Cell cycle Charcot-Marie-Tooth disease CMT1E Confocal microscopy Disease Doppler effect Genes Genetic aspects Genotype & phenotype Hemodynamics Hippocampus Homeostasis Life Sciences Microscope and microscopy Microscopy Mutation Myelin Nervous system Neuroimaging Peripheral myelin protein 22 Peripheral neuropathy Phenotypes Proteins Scanning Laser Confocal Microscopy Schwann cells Structure-function relationships Trembler-J Ultrafast Power Doppler Uruguay anxiety hippocampi behavioral tests Trembler-J Ultrafast Power Doppler CMT1E Scanning Laser Confocal Microscopy Ultrafast Power Doppler Scanning Laser Confocal Microscopy behavioral tests Trembler-J CMT1E anxiety hippocampi
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Summary:	The main human hereditary peripheral neuropathy (Charcot-Marie-Tooth, CMT), manifests in progressive sensory and motor deficits. Mutations in the compact myelin protein gene pmp22 cause more than 50% of all CMTs. CMT1E is a subtype of CMT1 myelinopathy carrying micro-mutations in pmp22. The Trembler-J mice have a spontaneous mutation in pmp22 identical to that present in CMT1E human patients. PMP22 is mainly (but not exclusively) expressed in Schwann cells. Some studies have found the presence of pmp22 together with some anomalies in the CNS of CMT patients. Recently, we identified the presence of higher hippocampal pmp22 expression and elevated levels of anxious behavior in TrJ/+ compared to those observed in wt. In the present paper, we delve deeper into the central expression of the neuropathy modeled in Trembler-J analyzing in vivo the cerebrovascular component by Ultrafast Doppler, exploring the vascular structure by scanning laser confocal microscopy, and analyzing the behavioral profile by anxiety and motor difficulty tests. We have found that TrJ/+ hippocampi have increased blood flow and a higher vessel volume compared with the wild type. Together with this, we found an anxiety-like profile in TrJ/+ and the motor difficulties described earlier. We demonstrate that there are specific cerebrovascular hemodynamics associated with a vascular structure and anxious behavior associated with the TrJ/+ clinical phenotype, a model of the human CMT1E disease.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	2079-7737 2079-7737
DOI:	10.3390/biology12101324