ArticleJournal of neurodevelopmental disorders2016
Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats.
Article in Journal of neurodevelopmental disorders, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 64 citations in OpenAlex.
- Vagus Nerve Stimulation Paired With Tones Alters the Auditory Cortex Proteome in a Rat Model of Rett Syndrome.Developmental neurobiology · 2026Article
- Ferroptosis is a novel pathogenic mechanism of FDXR-related disease via disruption of the NRF2 pathway.Cell death discovery · 2025Article
- Animal models of autism spectrum disorder: Insights into genetic, structural and environmental models.Veterinarni medicina · 2025Review
- Lifespan in rodents with MYT1L heterozygous mutation.Scientific reports · 2025Article
- Lifespan in rodents with MYT1L heterozygous mutation.Research square · 2024Article
- Animal Models of Autistic-like Behavior in Rodents: A Scoping Review and Call for a Comprehensive Scoring System.International journal of molecular sciences · 2024Article
- Article
- Understanding autism spectrum disorders with animal models: applications, insights, and perspectives.Zoological research · 2021Review
- Collapse of complexity of brain and body activity due to excessive inhibition and MeCP2 disruption.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Microbial community changes in a female rat model of Rett syndrome.Progress in neuro-psychopharmacology & biological psychiatry · 2021Article
- Reviewing Evidence for the Relationship of EEG Abnormalities and RTT Phenotype Paralleled by Insights from Animal Studies.International journal of molecular sciences · 2021Review
- Substantial acetylcholine reduction in multiple brain regions of Mecp2-deficient female rats and associated behavioral abnormalities.PloS one · 2021Article
- Review
- Deficits in skilled motor and auditory learning in a rat model of Rett syndrome.Journal of neurodevelopmental disorders · 2020Article
- Rat models of human diseases and related phenotypes: a systematic inventory of the causative genes.Journal of biomedical science · 2020Review
- Cerebral organoids as tools to identify the developmental roots of autism.Molecular autism · 2020Review
- A framework for the investigation of rare genetic disorders in neuropsychiatry.Nature medicine · 2019Review
- The role of MeCP2 in learning and memory.Learning & memory (Cold Spring Harbor, N.Y.) · 2019Article
- Treating Rett syndrome: from mouse models to human therapies.Mammalian genome : official journal of the International Mammalian Genome Society · 2019Review
- Mecp2 Disruption in Rats Causes Reshaping in Firing Activity and Patterns of Brainstem Respiratory Neurons.Neuroscience · 2019Article
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
backgroundRett Syndrome (RTT) is a neurodevelopmental disease caused by the disruption of the MECP2 gene. Several mouse models of RTT have been developed with Mecp2 disruptions. Although the mouse models are widely used in RTT research, results obtained need to be validated in other species. Therefore, we performed these studies to characterize phenotypes of a novel Mecp2 (-/Y) rat model and compared them with the Mecp2 (tm1.1Bird) mouse model of RTT.
methodsRTT-like phenotypes were systematically studied and compared between Mecp2 (-/Y) rats and Mecp2 (-/Y) mice. In-cage conditions of the rats were monitored. Grip strength and spontaneous locomotion were used to evaluate the motor function. Three-chamber test was performed to show autism-type behaviors. Breathing activity was recorded with the plethysmograph. Individual neurons in the locus coeruleus (LC) were studied in the whole-cell current clamp. The lifespan of the rats was determined with their survival time.
resultsMecp2 (-/Y) rats displayed growth retardation, malocclusion, and lack of movements, while hindlimb clasping was not seen. They had weaker forelimb grip strength and a lower rate of locomotion than the WT littermates. Defects in social interaction with other rats were obvious. Breathing frequency variation and apnea in the null rats were significantly higher than in the WT. LC neurons in the null rats showed excessive firing activity. A half of the null rats died in 2 months. Most of the RTT-like symptoms were comparable to those seen in Mecp2 (-/Y) mice, while some appeared more or less severe. The findings that most RTT-like symptoms exist in the rat model with moderate variations and differences from the mouse models support the usefulness of both Mecp2 (-/Y) rodent models.
conclusionsThe novel Mecp2 (-/Y) rat model recapitulated numerous RTT-like symptoms as Mecp2 (-/Y) mouse models did, which makes it a valuable alternative model in the RTT studies when the body size matters.
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