Behavioral and neurochemical characterization of the spontaneous mutation tremor, a new mouse model of audiogenic seizures

Garcia-Gomes, Mariana de Souza Aranha; Zanatto, Dennis Albert; Galvis-Alonso, Orfa Yineth; Mejía, Jorge; Antiorio, Ana Tada Fonseca Brasil; Yamamoto, Pedro Takao; Olivato, Márcia Carolina Millán; Sandini, Thaísa Meira; Flório, Jorge Camilo; Lebrun, Ivo; Massironi, Sílvia Maria Gomes; Alexandre-Ribeiro, Sandra Regina; Bernardi, Maria Martha; Ienne, Susan; Souza, Tiago Antônio de; Dagli, M.L.; Mori, Cláudia Madalena Cabrera

doi:10.1016/j.yebeh.2020.106945

Cited by 8 publications

(5 citation statements)

References 48 publications

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“…Egr3 is essential for nervous system development, particularly in the sympathetic autonomic nervous system [ 64 ]. Egr3 is also involved in motor coordination and motor skill learning functions [ 65 ]. The Adora2a gene’s polymorphism has been found to be linked to the occurrence of central nervous system disorders, including attention-deficit hyperactivity disorder (ADHD) and Tourette’s syndrome [ 66 , 67 ].…”

Section: Discussionmentioning

confidence: 99%

Identification of lncRNA-miRNA-mRNA Networks in the Lenticular Nucleus Region of the Brain Contributes to Hepatolenticular Degeneration Pathogenesis and Therapy

Hao,

Yang,

Yang

et al. 2023

Mol Neurobiol

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Long non-coding RNAs (lncRNAs) are a recently discovered group of non-coding RNAs that play a crucial role in the regulation of various human diseases, especially in the study of nervous system diseases which has garnered significant attention. However, there is limited knowledge on the identification and function of lncRNAs in hepatolenticular degeneration (HLD). The objective of this study was to identify novel lncRNAs and determine their involvement in the networks associated with HLD. We conducted a comprehensive analysis of RNA sequencing (RNA-seq) data, reverse transcription-quantitative polymerase chain reaction (RT-qPCR), and computational biology to identify novel lncRNAs and explore their potential mechanisms in HLD. We identified 212 differently expressed lncRNAs, with 98 upregulated and 114 downregulated. Additionally, 32 differently expressed mRNAs were found, with 15 upregulated and 17 downregulated. We obtained a total of 1131 pairs of co-expressed lncRNAs and mRNAs by Pearson correlation test and prediction and annotation of the lncRNA-targeted miRNA-mRNA network. The differential lncRNAs identified in this study were found to be involved in various biological functions and signaling pathways. These include translational initiation, motor learning, locomotors behavior, dioxygenase activity, integral component of postsynaptic membrane, neuroactive ligand-receptor interaction, nuclear factor-kappa B (NF-κB) signaling pathway, cholinergic synapse, sphingolipid signaling pathway, and Parkinson’s disease signaling pathway, as revealed by the Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analyses. Six lncRNAs, including XR_001782921.1 (P < 0.01), XR_ 001780581.1 (P < 0.01), ENSMUST_00000207119 (P < 0.01), XR_865512.2 (P < 0.01), TCONS_00005916 (P < 0.01), and TCONS_00020683 (P < 0.01), showed significant differences in expression levels between the model group and normal group by RT-qPCR. Among these, four lncRNAs (TCONS_00020683, XR_865512.2, XR_001780581.1, and ENSMUST00000207119) displayed a high degree of conservation. This study provides a unique perspective for the pathogenesis and therapy of HLD by constructing the lncRNA-miRNA-mRNA network. This insight provides a foundation for future exploration in this field.

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Section: Discussionmentioning

confidence: 99%

Identification of lncRNA-miRNA-mRNA Networks in the Lenticular Nucleus Region of the Brain Contributes to Hepatolenticular Degeneration Pathogenesis and Therapy

Hao,

Yang,

Yang

et al. 2023

Mol Neurobiol

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“…This strain is characterized not only by audiogenic generalized clonic seizures, but also by body tremor, ataxia, and decreased exploratory behavior. The tremor strain is characterized by increased expression (in comparison to wild type) of the Egr3 (early growth response protein 3) gene, which encodes a transcription factor controlling the expression of GABA A and NMDA receptors [ 83 , 210 , 211 ]. To date, we have been unable to find data on the association of mutations in this gene with human epilepsy.…”

Section: Different Models Of Audiogenic Epilepsy In Rodentsmentioning

confidence: 99%

“…Thus, increased expression levels of Egr3 may play a causative role in the development of AE in WAR and GASH/Sal as well. The AE-prone tremor mouse strain is also characterized by increased expression of the Egr3 gene [ 210 ]. On the other hand, we were unable to find data on the association of the Egr3 gene with human epilepsy.…”

Section: Different Models Of Audiogenic Epilepsy In Rodentsmentioning

confidence: 99%

“…As an example, Tsc1 and Tsc2 (Tuberous sclerosis complex) knockouts do not lead to the development of seizures in mice, unlike tuberous sclerosis cases in humans [ 293 , 294 ]. On the other hand, the opposite is also true, i.e., several mutations were described that induce seizures in rodents, and definite disorders in humans [ 83 , 210 , 211 ]. Pathological deviations that lead to epileptogenesis in AE-prone strains obtained by selection in independent experiments obviously do not coincide with one another because they could be determined by different mutations.…”

Section: Near-term Prospects and Conclusionmentioning

confidence: 99%

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Rodent Models of Audiogenic Epilepsy: Genetic Aspects, Advantages, Current Problems and Perspectives

et al. 2022

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Animal models of epilepsy are of great importance in epileptology. They are used to study the mechanisms of epileptogenesis, and search for new genes and regulatory pathways involved in the development of epilepsy as well as screening new antiepileptic drugs. Today, many methods of modeling epilepsy in animals are used, including electroconvulsive, pharmacological in intact animals, and genetic, with the predisposition for spontaneous or refractory epileptic seizures. Due to the simplicity of manipulation and universality, genetic models of audiogenic epilepsy in rodents stand out among this diversity. We tried to combine data on the genetics of audiogenic epilepsy in rodents, the relevance of various models of audiogenic epilepsy to certain epileptic syndromes in humans, and the advantages of using of rodent strains predisposed to audiogenic epilepsy in current epileptology.

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“…The seizure intensity in human patients and in experimental animals depends on the blood pH values and on the NMDA-receptors’ excitability [ 103 , 104 ]. The acidosis (low pH) was described as occurring during seizure process as well [ 105 , 106 ]. The analysis of the brain stem area “responsible” for respiration function was found to be different in WAR in comparison to controls [ 107 ].…”

Section: The Experimental Evidences Which Are Not Compatible With the Hypothesis Presentedmentioning

confidence: 99%

Rodent Brain Pathology, Audiogenic Epilepsy

et al. 2021

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The review presents data which provides evidence for the internal relationship between the stages of rodent audiogenic seizures and post-ictal catalepsy with the general pattern of animal reaction to the dangerous stimuli and/or situation. The wild run stage of audiogenic seizure fit could be regarded as an intense panic reaction, and this view found support in numerous experimental data. The phenomenon of audiogenic epilepsy probably attracted the attention of physiologists as rodents are extremely sensitive to dangerous sound stimuli. The seizure proneness in this group shares common physiological characteristics and depends on animal genotype. This concept could be the new platform for the study of epileptogenesis mechanisms.

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Behavioral and neurochemical characterization of the spontaneous mutation tremor, a new mouse model of audiogenic seizures

Cited by 8 publications

References 48 publications

Identification of lncRNA-miRNA-mRNA Networks in the Lenticular Nucleus Region of the Brain Contributes to Hepatolenticular Degeneration Pathogenesis and Therapy

Identification of lncRNA-miRNA-mRNA Networks in the Lenticular Nucleus Region of the Brain Contributes to Hepatolenticular Degeneration Pathogenesis and Therapy

Rodent Models of Audiogenic Epilepsy: Genetic Aspects, Advantages, Current Problems and Perspectives

Rodent Brain Pathology, Audiogenic Epilepsy

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