mecp2 - methyl-CpG binding protein 2 Details

Animal Model >> mecp2 Details

methyl CpG binding protein 2

Summary

Gene Symbol

mecp2

Gene Name

methyl CpG binding protein 2

Aliases

wu:fk96a04; zgc:111857

Model Species

Danio rerio

Model Type

Genetic

Model Summary

Knockdown of mecp2 in zebrafish results in decreased axonal and dendritic architecture, which is likely driven by decreased expression of axonal guidance genes sema3f, sema5b, and robo2. Mecp2 morphants show increased apoptosis. They also exhibit reduced response to tactile stimuli even though the muscle fiber morphology is not changed. Mecp2 null mutants likewise show a decrease in dendritic architecture and decreased expression of sema3f, sema5b, and robo2. These effects in morpholino-induced morphants could be rescued by injection of mecp2 mRNA and ameliorated by injection of sema3f, sema5b, or robo2 mRNA. Loss of mecp2 also results in initial growth retardation and deficits in gastrointestinal function. These are accompanied by decreased in innate immune response measured in the number of neutrophils, but no change in the number of microglia or macrophages is observed. Likewise, mecp2 loss results in profound loss of tnfa function, a proinflammatory cytokine. Transient changes in il1b and il10 are observed as well, suggestive of increased inflammatory signaling, which are followed by resolution of inflammation. Injection of mecp2 mRNA temporarily increases mecp2 expression, but this effect is only temporary. Injection of tnfa mRNA does not rescue the immune response or the initial reduced growth deficits in mecp2-null mutants. Moreover, mecp2-null mutants show abnormal gene expression profile, measured by RNA sequencing.

Number of Reports

Number of Models

113

External Links

ASD-associated variation model

Rett syndrome

ASD-associated variation model

Rett syndrome ASD

Animal Model >> mecp2 Details

methyl CpG binding protein 2

Summary

Gene Symbol

Mecp2

Gene Name

methyl CpG binding protein 2

Aliases

1500041B07Rik, D630021H01Rik, Mbd5, WBP10

Model Species

Mus musculus

Model Type

Genetic

Model Summary

Mecp2 duplication caused intellectual disability and autism; restoration of MeCP2 copy number in the adult abrogates the symptoms of autism.

Number of Reports

Number of Models

113

External Links

ASD-associated variation model

Rett syndrome

ASD-associated variation model

Rett syndrome ASD

Animal Model >> mecp2 Details

methyl CpG binding protein 2

Summary
Gene Symbol	Mecp2
Gene Name	methyl CpG binding protein 2
Aliases
Model Species	Rattus norvegicus
Model Type	Genetic
Model Summary	Mecp2 deficiency is sufficient to cause neuronal dysfunction with symptomatic manifestation similar to Rett syndrome.
Number of Reports	46
Number of Models	113
External Links
ASD-associated variation model	Rett syndrome
ASD-associated variation model	Rett syndrome ASD

References
Rett Syndrome, X-linked MR	Abnormalities of cell packing density and dendritic complexity in the MeCP2 A140V mouse model of Rett syndrome/X-linked mental retardation.	Jentarra GM , et al. 2010	20163734
Rett syndrome, ASD, juvenile-onset schizophrenia	Sensory integration in mouse insular cortex reflects GABA circuit maturation.	Gogolla N , et al. 2014	25088363
Rett syndrome, ASD, juvenile-onset schizophrenia	Cerebellar associative sensory learning defects in five mouse autism models.	Kloth AD , et al. 2015	26158416
Rett Syndrome	A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome.	Guy J , et al. 2001	11242117
Rett Syndrome	Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice.	Chen RZ , et al. 2001	11242118
Rett Syndrome	Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3.	Shahbazian M , et al. 2002	12160743
Rett Syndrome	Delayed maturation of neuronal architecture and synaptogenesis in cerebral cortex of Mecp2-deficient mice.	Fukuda T , et al. 2005	15977646
Rett Syndrome	Mecp2 deficiency is associated with learning and cognitive deficits and altered gene activity in the hippocampal region of mice.	Pelka GJ , et al. 2006	16467389
Rett Syndrome	Brain-derived neurotrophic factor expression and respiratory function improve after ampakine treatment in a mouse model of Rett syndrome.	Ogier M , et al. 2007	17913925
Rett Syndrome	Progressive noradrenergic deficits in the locus coeruleus of Mecp2 deficient mice.	Roux JC , et al. 2009	19998492
Rett Syndrome	Exogenous brain-derived neurotrophic factor rescues synaptic dysfunction in Mecp2-null mice.	Kline DD , et al. 2010	20392952
Rett Syndrome	Rett syndrome mutation MeCP2 T158A disrupts DNA binding, protein stability and ERP responses.	Goffin D , et al. 2011	22119903
Rett Syndrome	Peripheral Mechanosensory Neuron Dysfunction Underlies Tactile and Behavioral Deficits in Mouse Models of ASDs.	Orefice LL , et al. 2016	27293187
Rett Syndrome	D-cycloserine improves synaptic transmission in an animal mode of Rett syndrome.	Na ES , et al. 2017	28813484
Rett syndrome	Radically truncated MeCP2 rescues Rett syndrome-like neurological defects.	Tillotson R , et al. 2017	29019980
Primary	Degraded neural and behavioral processing of speech sounds in a rat model of Rett syndrome.	Engineer CT , et al. 2015	26321676
Primary	Methyl-CpG Binding Protein 2 (Mecp2) Regulates Sensory Function Through Sema5b and Robo2.	Leong WY , et al. 2016	26733807
ASD	Mild overexpression of MeCP2 causes a progressive neurological disorder in mice.	Collins AL , et al. 2004	15351775
ASD	Crh and Oprm1 mediate anxiety-related behavior and social approach in a mouse model of MECP2 duplication syndrome.	Samaco RC , et al. 2012	22231481
ASD	Loss of MeCP2 in Parvalbumin-and Somatostatin-Expressing Neurons in Mice Leads to Distinct Rett Syndrome-like Phenotypes.	Ito-Ishida A , et al. 2015	26590342
ASD	Reversal of phenotypes in MECP2 duplication mice using genetic rescue or antisense oligonucleotides.	Sztainberg Y , et al. 2015	26605526
ASD	Peripheral Mechanosensory Neuron Dysfunction Underlies Tactile and Behavioral Deficits in Mouse Models of ASDs.	Orefice LL , et al. 2016	27293187
Additional	Characterization of Rett Syndrome-like phenotypes in Mecp2-knockout rats.	Wu Y , et al. 2016	27313794
Additional	MeCP2 deficiency results in robust Rett-like behavioural and motor deficits in male and female rats.	Patterson KC , et al. 2016	27329765
Additional	Loss of MeCP2 in the rat models regression, impaired sociability and transcriptional deficits of Rett syndrome.	Veeraragavan S , et al. 2016	27365498
Additional	Neuronal cytoskeletal gene dysregulation and mechanical hypersensitivity in a rat model of Rett syndrome.	Bhattacherjee A , et al. 2017	28760966
Additional	Mecp2 regulates tnfa during zebrafish embryonic development and acute inflammation.	van der Vaart M , et al. 2017	28993314
Additional	Motor, Somatosensory, Viscerosensory and Metabolic Impairments in a Heterozygous Female Rat Model of Rett Syndrome.	Bhattacherjee A , et al. 2017	29286317
Additional	Striatal Inhibition of MeCP2 or TSC1 Produces Sociability Deficits and Repetitive Behaviors.	Lee Y , et al. 2019	30636904
Additional	Targeting Peripheral Somatosensory Neurons to Improve Tactile-Related Phenotypes in ASD Models.	Orefice LL , et al. 2019	31398341
Additional	Locus-specific DNA Methylation of Mecp2 Promoter Leads to Autism-Like Phenotypes in Mice		32015323
Additional	Presymptomatic training mitigates functional deficits in a mouse model of Rett syndrome	Achilly NP et al. 2021	33762729
Additional	Inhibition of Elevated Ras-MAPK Signaling Normalizes Enhanced Motor Learning and Excessive Clustered Dendritic Spine Stabilization in the MECP2-Duplication Syndrome Mouse Model of Autism	Ash RT et al. 2021	34021030
Additional		et al.	38233682
	Phosphorylation of MeCP2 at Serine 80 regulates its chromatin association and neurological function.	Tao J , et al. 2009	19225110
	Synaptic circuit abnormalities of motor-frontal layer 2/3 pyramidal neurons in an RNA interference model of methyl-CpG-binding protein 2 deficiency.	Wood L , et al. 2009	19812320
	Loss of MeCP2 in aminergic neurons causes cell-autonomous defects in neurotransmitter synthesis and specific behavioral abnormalities.	Samaco RC , et al. 2009	20007372
	Synaptic circuit abnormalities of motor-frontal layer 2/3 pyramidal neurons in a mutant mouse model of Rett syndrome.	Wood L and Shepherd GM 2010	20138994
	Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes.	Chao HT , et al. 2010	21068835
	Modification of Mecp2 dosage alters axonal transport through the Huntingtin/Hap1 pathway.	Roux JC , et al. 2011	22127389
	Pharmacological interference with the glucocorticoid system influences symptoms and lifespan in a mouse model of Rett syndrome.	Braun S , et al. 2011	22186023
	7,8-dihydroxyflavone exhibits therapeutic efficacy in a mouse model of Rett syndrome.	Johnson RA , et al. 2011	22194327
	Crh and Oprm1 mediate anxiety-related behavior and social approach in a mouse model of MECP2 duplication syndrome.	Samaco RC , et al. 2012	22231481
	MeCP2 mouse model of RTT reproduces auditory phenotypes associated with Rett syndrome and replicate select EEG endophenotypes of autism spectrum...	Liao W , et al. 2012	22249109
	Long-lived epigenetic interactions between perinatal PBDE exposure and Mecp2308 mutation.	Woods R , et al. 2012	22343140
	A mouse model for MeCP2 duplication syndrome: MeCP2 overexpression impairs learning and memory and synaptic transmission.	Na ES , et al. 2012	22378884
	Female Mecp2() mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies.	Samaco RC , et al. 2012	23026749
	Acetyl-L-carnitine improves behavior and dendritic morphology in a mouse model of Rett syndrome.	Schaevitz LR , et al. 2012	23227269

Various Models
	MECP2_4_KO_HE_TRUNC(308) et al.	MECP2_4_KO_HT_TRUNC(308) et al.	M_MECP2_1.2_CKO_HE et al.	M_MECP2_1.2_KO_HE et al.	M_MECP2_1.2_KO_HE_AMPAKINE et al.
	M_MECP2_1.3_CKO_HE et al.	M_MECP2_1.3_KO_HE et al.	M_MECP2_1.3_KO_HE_ALC et al.	M_MECP2_1.3_KO_HE_CYCLOSERINE et al.	M_MECP2_1.3_KO_HE_DHF et al.
	M_MECP2_1.3_KO_HT et al.	M_MECP2_1.3_KO_HT_ALC et al.	M_MECP2_1.4_CKO_HE et al.	M_MECP2_10_PME_HIPPOCAMPUS	M_MECP2_11_KO_HE et al.
	M_MECP2_14_KI_HE_A140V et al.	M_MECP2_14_KI_HT_A140V et al.	M_MECP2_1_CKO_HE et al.	M_MECP2_1_KO_HE et al.	M_MECP2_20_KI_HE_T158A et al.
	M_MECP2_20_KI_HT_T158A et al.	M_MECP2_23_KI_HT_TRUNC(308)_BDE47 et al.	M_MECP2_26_OE_HE_NEURON-WB et al.	M_MECP2_34.1_TG_2X et al.	M_MECP2_34.1_TG_2X_ANTALARMIN et al.
	M_MECP2_34.1_TG_2X_CRHKO et al.	M_MECP2_34.1_TG_2X_CRHR1KO et al.	M_MECP2_34.2_TG_2X et al.	M_MECP2_34.2_TG_2X_OPRM1KO et al.	M_MECP2_34_TG_2X et al.
	M_MECP2_36.1_TG_7X et al.	M_MECP2_36.1_TG_7X_ASO et al.	M_MECP2_36.2_TG_7X et al.	M_MECP2_36_TG_7X et al.	M_MECP2_39_KI_HE_R306C et al.
	M_MECP2_40_KI_HE_STOP et al.	M_MECP2_40_KI_HE_STOP_REINSTATE-MECP2 et al.	M_MECP2_46_KI_HE_TRUNC(N-TERM) et al.	M_MECP2_47_KI_HE_TRUNC(N-C-TERM) et al.	M_MECP2_48_KI_HE_TRUNC(N-I-C) et al.
	M_MECP2_49_KI_HE_R133C et al.	M_MECP2_4_KI_HE_TRUNC(308) et al.	M_MECP2_4_KI_HT_TRUNC(308) et al.	M_MECP2_5.11_CKO_HE et al.	M_MECP2_5.12_CKO_HE et al.
	M_MECP2_5.13_CKO_HE et al.	M_MECP2_5.13_CKO_HT et al.	M_MECP2_5.14_CKO_HE et al.	M_MECP2_5.15_CKO_HE et al.	M_MECP2_5.15_CKO_HT et al.
	M_MECP2_5.2_KO_HE et al.	M_MECP2_5.2_KO_HE_CORTISONE-H et al.	M_MECP2_5.2_KO_HE_CORTISONE-L et al.	M_MECP2_5.2_KO_HE_CYSTEAMINE et al.	M_MECP2_5.2_KO_HE_RU486 et al.
	M_MECP2_5.2_KO_HT_CORTISONE-H et al.	M_MECP2_5.2_KO_HT_CORTISONE-L et al.	M_MECP2_5.2_KO_HT_RU486 et al.	M_MECP2_5.3_KO_HT et al.	M_MECP2_5.4_KO_HT et al.
	M_MECP2_5.5_CKO_HE_PVALBN et al.	M_MECP2_5.6_CKO_HE_SSTN et al.	M_MECP2_5.7_CKO_HE et al.	M_MECP2_5.8_CKO_HE et al.	M_MECP2_5.9_CKO_HE et al.
	M_MECP2_50_KI_HE_STOP et al.	M_MECP2_50_KI_HE_STOP_REINSTATE-DNIC et al.	M_MECP2_51_CKO_HT Orefice LL , et al. 2019	M_MECP2_52_CKO_HE Orefice LL , et al. 2019	M_MECP2_52_CKO_HE_ISOGUVACINE Orefice LL , et al. 2019
	M_MECP2_52_CKO_HE_MIDAZOLAM Orefice LL , et al. 2019	M_MECP2_53_KO_HE Orefice LL , et al. 2019	M_MECP2_53_KO_HE_REINSTATEMENT-ADVILLIN Orefice LL , et al. 2019	M_MECP2_53_KO_HE_REINSTATEMENT-ADVILLIN-ICRE-1 Orefice LL , et al. 2019	M_MECP2_54_CKO_HE Orefice LL , et al. 2019
	M_MECP2_55_CKO_HE Orefice LL , et al. 2019	M_MECP2_56_CKO_HE Orefice LL , et al. 2019	M_MECP2_57_KI_HE Orefice LL , et al. 2019	M_MECP2_57_KI_HE_GABRB3-CRE Orefice LL , et al. 2019	M_MECP2_57_KI_HE_ISOGUVACINE Orefice LL , et al. 2019
	M_MECP2_58_KI_HT Orefice LL , et al. 2019	M_MECP2_58_KI_HT_GABRB3-CRE Orefice LL , et al. 2019	M_MECP2_58_KI_HT_ISOGUVACINE Orefice LL , et al. 2019	M_MECP2_59_TG_2X Ash RT et al. 2021	M_MECP2_59_TG_2X_SL327 Ash RT et al. 2021
	M_MECP2_5_KO_HE et al.	M_MECP2_5_KO_HE_HDNIC et al.	M_MECP2_5_KO_HT Achilly NP et al. 2021	M_MECP2_5_KO_HT_TRAINING Achilly NP et al. 2021	M_MECP2_6_KD Lee Y , et al. 2019
	M_MECP2_6_KD_CYCLOSERINE Lee Y , et al. 2019	M_MECP2_6_KD_FENOBAM Lee Y , et al. 2019	M_MECP2_6_KD_SCH-23390 Lee Y , et al. 2019	M_MECP2_6_KD_SCH-39166 Lee Y , et al. 2019	M_MECP2_7_KI_HE_S80A et al.
	M_MECP2_8_CKD_HM_PYRAMIDALN-L2-3 et al.	M_MECP2_9_PME	R_MECP2_1_KO_HT Engineer CT , et al. 2015	R_MECP2_2_KO_HE Bhattacherjee A , et al. 2017 Veeraragavan S , et al. 2016 Patterson KC , et al. 2016 Wu Y , et al. 2016	R_MECP2_3_KD Bhattacherjee A , et al. 2017
	Z_MECP2_1_KD_MO Leong WY , et al. 2016	Z_MECP2_1_KD_MO_HUC-MECP2 Leong WY , et al. 2016	Z_MECP2_1_KD_MO_MECP2 Leong WY , et al. 2016	Z_MECP2_1_KD_MO_ROBO-SEMA5B Leong WY , et al. 2016	Z_MECP2_1_KD_MO_ROBO2 Leong WY , et al. 2016
	Z_MECP2_1_KD_MO_SEMA5B Leong WY , et al. 2016	Z_MECP2_2_KO_HM Leong WY , et al. 2016	Z_MECP2_2_KO_HM_MECP2-1 van der Vaart M , et al. 2017	Z_MECP2_2_KO_HM_MECP2-2 van der Vaart M , et al. 2017	Z_MECP2_2_KO_HM_MECP2_MO Leong WY , et al. 2016
	Z_MECP2_2_KO_HM_TNFA van der Vaart M , et al. 2017	Z_MECP2_3_KO_HT van der Vaart M , et al. 2017	Z_MECP2_4_KD_MO van der Vaart M , et al. 2017


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methyl CpG binding protein 2

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