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Central Alteration in Peripheral Neuropathy of Trembler-J Mice: Hippocampal pmp22 Expression and Behavioral Profile in Anxiety Tests

dc.contributor.authorJuan Pablo Damián
dc.contributor.authorLucia Vázquez Alberdi
dc.contributor.authorLucía Canclini
dc.contributor.authorGonzalo Rosso
dc.contributor.authorSilvia Olivera Bravo
dc.contributor.authorMariana Martínez
dc.contributor.authorNatalia Uriarte
dc.contributor.authorPaul Ruiz
dc.contributor.authorMiguel Calero
dc.contributor.authorMaría Vittoria Di Tomaso
dc.contributor.authorAlejandra Kun
dc.contributor.otherUnidad de Bioquímica, Departamento de Biociencias Veterinarias, Facultad de Veterinaria, Universidad de la República, 11600 Montevideo, Uruguay
dc.contributor.otherDepartamento de Proteínas y Ácidos Nucleicos, Instituto de Investigaciones Biológicas Clemente Estable, 11600 Montevideo, Uruguay
dc.contributor.otherDepartamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, 11600 Montevideo, Uruguay
dc.contributor.otherMax Planck Institute for the Science of Light, Max-Planck-Zentrum für Physik und Medizin, 91058 Erlangen, Germany
dc.contributor.otherNeurobiología Celular y Molecular, Instituto de Investigaciones Biológicas Clemente Estable, 11600 Montevideo, Uruguay
dc.contributor.otherDepartamento de Proteínas y Ácidos Nucleicos, Instituto de Investigaciones Biológicas Clemente Estable, 11600 Montevideo, Uruguay
dc.contributor.otherLaboratorio de Neurociencias, Facultad de Ciencias, Universidad de la República, 11400 Montevideo, Uruguay
dc.contributor.otherUnidad de Biofísica, Departamento de Biociencias Veterinarias, Facultad de Veterinaria, Universidad de la República, 11600 Montevideo, Uruguay
dc.contributor.otherUnidad de Encefalopatías Espongiformes, UFIEC, CIBERNED, CIEN Foundation, Queen Sofia Foundation Alzheimer Center, Instituto de Salud Carlos III, 28031 Madrid, Spain
dc.contributor.otherDepartamento de Genética, Instituto de Investigaciones Biológicas Clemente Estable, 11600 Montevideo, Uruguay
dc.contributor.otherDepartamento de Proteínas y Ácidos Nucleicos, Instituto de Investigaciones Biológicas Clemente Estable, 11600 Montevideo, Uruguay
dc.date.accessioned2025-10-09T05:17:00Z
dc.date.available2025-10-09T05:17:00Z
dc.date.issued01-04-2021
dc.identifier.urihttps://www.mdpi.com/2218-273X/11/4/601
dc.identifier.urihttp://digilib.fisipol.ugm.ac.id/repo/handle/15717717/40913
dc.description.abstractCharcot–Marie–Tooth (CMT) type 1 disease is the most common human hereditary demyelinating neuropathy. Mutations in pmp22 cause about 70% of all CMT1. Trembler-J (TrJ/+) mice are an animal model of CMT1E, having the same spontaneous pmp22 mutation that is found in humans. We compared the behavior profile of TrJ/+ and +/+ (wild-type) in open-field and elevated-plus-maze anxiety tests. In these tests, TrJ/+ showed an exclusive head shake movement, a lower frequency of rearing, but a greater frequency of grooming. In elevated-plus-maze, TrJ/+ defecate more frequently, performed fewer total entries, and have fewer entries to closed arms. These hippocampus-associated behaviors in TrJ/+ are consistent with increased anxiety levels. The expression of pmp22 and soluble PMP22 were evaluated in E17-hippocampal neurons and adult hippocampus by in situ hybridization and successive immunohistochemistry. Likewise, the expression of pmp22 was confirmed by RT-qPCR in the entire isolated hippocampi of both genotypes. Moreover, the presence of aggregated PMP22 was evidenced in unmasked granular hippocampal adult neurons and shows genotypic differences. We showed for the first time a behavior profile trait associated with anxiety and a differential expression of pmp22/PMP22 in hippocampal neurons of TrJ/+ and +/+ mice, demonstrating the involvement at the central level in an animal model of peripheral neuropathy (CMT1E).
dc.language.isoEN
dc.publisherMDPI AG
dc.subject.lccMicrobiology
dc.titleCentral Alteration in Peripheral Neuropathy of Trembler-J Mice: Hippocampal pmp22 Expression and Behavioral Profile in Anxiety Tests
dc.typeArticle
dc.description.keywordsCharcot–Marie–Tooth
dc.description.keywordshippocampus
dc.description.keywordsperipheral-myelin-protein-22
dc.description.keywordsanxiety
dc.description.keywordsTrembler-J
dc.description.keywordsCA3 neurons
dc.description.doi10.3390/biom11040601
dc.title.journalBiomolecules
dc.identifier.e-issn2218-273X
dc.identifier.oaioai:doaj.org/journal:461983feae5e491e80584f460915064d
dc.journal.infoVolume 11, Issue 4


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