RGD Reference Report - Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats. - Rat Genome Database

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Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats.

Authors: Wu, Y  Zhong, W  Cui, N  Johnson, CM  Xing, H  Zhang, S  Jiang, C 
Citation: Wu Y, etal., J Neurodev Disord. 2016 Jun 16;8:23. doi: 10.1186/s11689-016-9156-7. eCollection 2016.
RGD ID: 11568037
Pubmed: PMID:27313794   (View Abstract at PubMed)
PMCID: PMC4910223   (View Article at PubMed Central)
DOI: DOI:10.1186/s11689-016-9156-7   (Journal Full-text)

BACKGROUND: Rett 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. METHODS: RTT-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. RESULTS: Mecp2 (-/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. CONCLUSIONS: The 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.

RGD Manual Disease Annotations    Click to see Annotation Detail View

Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
MECP2HumanRett syndrome  ISOMecp2 (Rattus norvegicus) RGD 
Mecp2RatRett syndrome  IMP  RGD 
Mecp2MouseRett syndrome  ISOMecp2 (Rattus norvegicus) RGD 
Mecp2em1SageRatRett syndrome  IMP  RGD 
SD-Mecp2em1SageRatRett syndrome  IMP  RGD 

Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process


Phenotype Annotations    Click to see Annotation Detail View

Mammalian Phenotype

Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
Mecp2Ratabnormal action potential  IMP  RGD 
Mecp2em1SageRatabnormal action potential  IMP  RGD 
SD-Mecp2em1SageRatabnormal action potential  IMP  RGD 
Mecp2Ratabnormal pulmonary respiratory rate  IMP  RGD 
Mecp2em1SageRatabnormal pulmonary respiratory rate  IMP  RGD 
SD-Mecp2em1SageRatabnormal pulmonary respiratory rate  IMP  RGD 
Mecp2Ratabnormal response to social novelty  IMP  RGD 
Mecp2em1SageRatabnormal response to social novelty  IMP  RGD 
SD-Mecp2em1SageRatabnormal response to social novelty  IMP  RGD 
Mecp2Ratdecreased body height  IMP  RGD 
Mecp2em1SageRatdecreased body height  IMP  RGD 
SD-Mecp2em1SageRatdecreased body height  IMP  RGD 
Mecp2Ratdecreased body weight  IMP  RGD 
Mecp2em1SageRatdecreased body weight  IMP  RGD 
SD-Mecp2em1SageRatdecreased body weight  IMP  RGD 
Mecp2Ratdecreased grip strength  IMP  RGD 
Mecp2em1SageRatdecreased grip strength  IMP  RGD 
SD-Mecp2em1SageRatdecreased grip strength  IMP  RGD 
Mecp2Ratdecreased locomotor activity  IMP  RGD 
Mecp2em1SageRatdecreased locomotor activity  IMP  RGD 
SD-Mecp2em1SageRatdecreased locomotor activity  IMP  RGD 
Mecp2Rathyperpnea  IMP  RGD 
Mecp2em1SageRathyperpnea  IMP  RGD 
SD-Mecp2em1SageRathyperpnea  IMP  RGD 
Mecp2Ratmalocclusion  IMP  RGD 
Mecp2em1SageRatmalocclusion  IMP  RGD 
SD-Mecp2em1SageRatmalocclusion  IMP  RGD 
Mecp2Ratpenis prolapse  IMP  RGD 
Mecp2em1SageRatpenis prolapse  IMP  RGD 
SD-Mecp2em1SageRatpenis prolapse  IMP  RGD 
Objects Annotated

Genes (Rattus norvegicus)
Mecp2  (methyl CpG binding protein 2)
Mecp2em1Sage  (methyl CpG binding protein 2; zinc finger nuclease induced mutant 1, Sigma Advanced Genetic Engineering Labs)

Genes (Mus musculus)
Mecp2  (methyl CpG binding protein 2)

Genes (Homo sapiens)
MECP2  (methyl-CpG binding protein 2)

SD-Mecp2em1Sage  (NA)

Additional Information