Reactome | Transcriptional Regulation by MECP2

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Transcriptional Regulation by MECP2 Show undefined attributes

authored	[InstanceEdit:9024208] Orlic-Milacic, Marija, 2017-10-02
created	[InstanceEdit:8986945] Orlic-Milacic, Marija, 2017-05-05
dbId	8986944
diseasePathways	[Pathway:R-HSA-9022534] Loss of MECP2 binding ability to 5hmC-DNA - Homo sapiens [Pathway:R-HSA-9022535] Loss of phosphorylation of MECP2 at T308 - Homo sapiens [Pathway:R-HSA-9022538] Loss of MECP2 binding ability to 5mC-DNA - Homo sapiens [Pathway:R-HSA-9022537] Loss of MECP2 binding ability to the NCoR/SMRT complex - Homo sapiens
displayName	Transcriptional Regulation by MECP2
doi	10.3180/R-HSA-8986944.1
edited	[InstanceEdit:9615799] Orlic-Milacic, Marija, 2018-08-08
eventOf	[Pathway:R-HSA-212436] Generic Transcription Pathway - Homo sapiens
goBiologicalProcess	[GO_BiologicalProcess:2829] epigenetic regulation of gene expression
hasDiagram	true
hasEHLD	false
hasEvent	[Pathway:R-HSA-9022692] Regulation of MECP2 expression and activity - Homo sapiens [Reaction:R-HSA-9022456] MECP2 binds 5mC-DNA - Homo sapiens [Reaction:R-HSA-9022453] MECP2 binds 5hmC-DNA - Homo sapiens [Reaction:R-HSA-9022315] MECP2 binds DGCR8 - Homo sapiens [Pathway:R-HSA-9022699] MECP2 regulates neuronal receptors and channels - Homo sapiens [Pathway:R-HSA-9022702] MECP2 regulates transcription of neuronal ligands - Homo sapiens [Pathway:R-HSA-9022707] MECP2 regulates transcription factors - Homo sapiens [Pathway:R-HSA-9022927] MECP2 regulates transcription of genes involved in GABA signaling - Homo sapiens [Reaction:R-HSA-9006503] MECP2 binds the PVALB gene promoter - Homo sapiens [BlackBoxEvent:R-HSA-9006508] PVALB gene expression is repressed by MECP2 - Homo sapiens [Reaction:R-HSA-9021945] MECP2 binds GAMT gene promoter - Homo sapiens [BlackBoxEvent:R-HSA-9021953] GAMT gene expression is stimulated by MECP2 - Homo sapiens [Reaction:R-HSA-9022770] MECP2 binds SGK1 gene promoter - Homo sapiens [BlackBoxEvent:R-HSA-9022764] SGK1 gene expression is inhibited by MECP2 - Homo sapiens [Reaction:R-HSA-9022872] MECP2 binds MOBP gene promoter - Homo sapiens [BlackBoxEvent:R-HSA-9022870] MOBP gene expression is inhibited by MECP2 - Homo sapiens [Reaction:R-HSA-9615536] MECP2 and SOX2 bind MIR137 gene - Homo sapiens [BlackBoxEvent:R-HSA-9615554] MIR137 gene expression is inhibited by MECP2 and SOX2 - Homo sapiens [Reaction:R-HSA-9615570] miR-137 binds PTEN mRNA - Homo sapiens [BlackBoxEvent:R-HSA-9615571] PTEN mRNA translation is inhibited by miR-137 - Homo sapiens [Reaction:R-HSA-9615801] MECP2 binds IRAK1 gene - Homo sapiens [BlackBoxEvent:R-HSA-9615829] IRAK1 gene expression is inhibited by MECP2 - Homo sapiens
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:9006983] Phosphorylation of MeCP2 at Serine 80 regulates its chromatin association and neurological function [LiteratureReference:9017785] MeCP2 deficiency disrupts axonal guidance, fasciculation, and targeting by altering Semaphorin 3F function [LiteratureReference:9020662] GluD1 is a common altered player in neuronal differentiation from both MECP2-mutated and CDKL5-mutated iPS cells [LiteratureReference:9005560] A previously unidentified MECP2 open reading frame defines a new protein isoform relevant to Rett syndrome [LiteratureReference:9020925] Developmental Dynamics of Rett Syndrome [LiteratureReference:9020924] Over-expression of either MECP2_e1 or MECP2_e2 in neuronally differentiated cells results in different patterns of gene expression [LiteratureReference:9020953] N-CoR mediates DNA methylation-dependent repression through a methyl CpG binding protein Kaiso [LiteratureReference:9020954] The major form of MeCP2 has a novel N-terminus generated by alternative splicing [LiteratureReference:9011208] Methyl-CpG Binding Protein 2 (Mecp2) Regulates Sensory Function Through Sema5b and Robo2 [LiteratureReference:9021710] Brahma links the SWI/SNF chromatin-remodeling complex with MeCP2-dependent transcriptional silencing [LiteratureReference:9021708] Testing for association between MeCP2 and the brahma-associated SWI/SNF chromatin-remodeling complex [LiteratureReference:9021806] Methyl-CpG-binding protein, MeCP2, is a target molecule for maintenance DNA methyltransferase, Dnmt1 [LiteratureReference:9021089] MeCP2, a key contributor to neurological disease, activates and represses transcription [LiteratureReference:9021892] Creb1-Mecp2-(m)CpG complex transactivates postnatal murine neuronal glucose transporter isoform 3 expression [LiteratureReference:9022244] Global transcriptional and translational repression in human-embryonic-stem-cell-derived Rett syndrome neurons [LiteratureReference:9022554] MECP2 promoter methylation and X chromosome inactivation in autism [LiteratureReference:9022565] Differential distribution of the MeCP2 splice variants in the postnatal mouse brain [LiteratureReference:9022572] Isoform-specific anti-MeCP2 antibodies confirm that expression of the e1 isoform strongly predominates in the brain [LiteratureReference:9022575] Brain region-specific expression of MeCP2 isoforms correlates with DNA methylation within Mecp2 regulatory elements [LiteratureReference:9022587] Mice with an isoform-ablating Mecp2 exon 1 mutation recapitulate the neurologic deficits of Rett syndrome [LiteratureReference:9022592] Decitabine alters the expression of Mecp2 isoforms via dynamic DNA methylation at the Mecp2 regulatory elements in neural stem cells [LiteratureReference:9022596] Epigenetic transmission of the impact of early stress across generations [LiteratureReference:9022813] Reversal of neurological defects in a mouse model of Rett syndrome [LiteratureReference:9022816] Mild overexpression of MeCP2 causes a progressive neurological disorder in mice [LiteratureReference:9022814] Expression of MeCP2 in postmitotic neurons rescues Rett syndrome in mice [LiteratureReference:9022810] MECP2 Duplication Syndrome [LiteratureReference:9022830] Duplication of the MECP2 region is a frequent cause of severe mental retardation and progressive neurological symptoms in males [LiteratureReference:9022837] Partial reversal of Rett Syndrome-like symptoms in MeCP2 mutant mice [LiteratureReference:9022845] Reduced AKT/mTOR signaling and protein synthesis dysregulation in a Rett syndrome animal model [LiteratureReference:9022858] Reversal of phenotypes in MECP2 duplication mice using genetic rescue or antisense oligonucleotides [LiteratureReference:9023074] A role for glia in the progression of Rett's syndrome [LiteratureReference:9023081] Rett syndrome microglia damage dendrites and synapses by the elevated release of glutamate [LiteratureReference:9023089] Transgenic complementation of MeCP2 deficiency: phenotypic rescue of Mecp2-null mice by isoform-specific transgenes [LiteratureReference:9023676] Mutations in MECP2 exon 1 in classical Rett patients disrupt MECP2_e1 transcription, but not transcription of MECP2_e2 [LiteratureReference:9022938] Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes [LiteratureReference:9006491] NMDA receptor regulation prevents regression of visual cortical function in the absence of Mecp2 [LiteratureReference:8986952] Activity-dependent phosphorylation of MeCP2 threonine 308 regulates interaction with NCoR [LiteratureReference:9023062] Conditional deletion of Mecp2 in parvalbumin-expressing GABAergic cells results in the absence of critical period plasticity [LiteratureReference:9017905] Epigenetic programming of mu-opioid receptor gene in mouse brain is regulated by MeCP2 and Brg1 chromatin remodelling factor [LiteratureReference:9017945] Up-regulation of the mu-opioid receptor gene is mediated through chromatin remodeling and transcriptional factors in differentiated neuronal cells [LiteratureReference:9023684] IUGR differentially alters MeCP2 expression and H3K9Me3 of the PPAR? gene in male and female rat lungs during alveolarization [LiteratureReference:9022797] Activity-dependent neuronal signalling and autism spectrum disorder [LiteratureReference:9023580] Transcriptional Regulation of Brain-Derived Neurotrophic Factor (BDNF) by Methyl CpG Binding Protein 2 (MeCP2): a Novel Mechanism for Re-Myelination and/or Myelin Repair Involved in the Treatment of Multiple Sclerosis (MS) [LiteratureReference:9023536] PTP1B inhibition suggests a therapeutic strategy for Rett syndrome [LiteratureReference:9006006] Activity-dependent BDNF release and TRPC signaling is impaired in hippocampal neurons of Mecp2 mutant mice [LiteratureReference:9023137] Cell cycle-linked MeCP2 phosphorylation modulates adult neurogenesis involving the Notch signalling pathway [LiteratureReference:9005556] Rett syndrome mutations abolish the interaction of MeCP2 with the NCoR/SMRT co-repressor [LiteratureReference:9017461] MeCP2 controls an epigenetic pathway that promotes myofibroblast transdifferentiation and fibrosis [LiteratureReference:9017985] Enhanced anxiety and stress-induced corticosterone release are associated with increased Crh expression in a mouse model of Rett syndrome [LiteratureReference:9005993] Transcriptional repression by the methyl-CpG-binding protein MeCP2 involves a histone deacetylase complex [LiteratureReference:9022718] Up-regulation of glucocorticoid-regulated genes in a mouse model of Rett syndrome [LiteratureReference:8986955] Transcriptional regulation of the MET receptor tyrosine kinase gene by MeCP2 and sex-specific expression in autism and Rett syndrome [LiteratureReference:9022360] The Rett syndrome protein MeCP2 regulates synaptic scaling [LiteratureReference:9017942] Crh and Oprm1 mediate anxiety-related behavior and social approach in a mouse model of MECP2 duplication syndrome [LiteratureReference:9022719] Mecp2-null mice provide new neuronal targets for Rett syndrome [LiteratureReference:9006581] Deletion of protein tyrosine phosphatase, non-receptor type 4 (PTPN4) in twins with a Rett syndrome-like phenotype [LiteratureReference:9022310] MeCP2 suppresses nuclear microRNA processing and dendritic growth by regulating the DGCR8/Drosha complex [LiteratureReference:9023483] miR-199a Links MeCP2 with mTOR Signaling and Its Dysregulation Leads to Rett Syndrome Phenotypes [LiteratureReference:9023577] MeCP2 binds to non-CG methylated DNA as neurons mature, influencing transcription and the timing of onset for Rett syndrome [LiteratureReference:9022452] MeCP2 binds to 5hmC enriched within active genes and accessible chromatin in the nervous system [LiteratureReference:9021097] BDNF deregulation in Rett syndrome
maxDepth	3
modified	[InstanceEdit:10651445] Weiser, Joel, 2026-03-16
name	Transcriptional Regulation by MECP2
orthologousEvent	[Pathway:R-XTR-8986944] Transcriptional Regulation by MECP2 - Xenopus tropicalis [Pathway:R-DRE-8986944] Transcriptional Regulation by MECP2 - Danio rerio [Pathway:R-SSC-8986944] Transcriptional Regulation by MECP2 - Sus scrofa [Pathway:R-BTA-8986944] Transcriptional Regulation by MECP2 - Bos taurus [Pathway:R-CFA-8986944] Transcriptional Regulation by MECP2 - Canis familiaris [Pathway:R-RNO-8986944] Transcriptional Regulation by MECP2 - Rattus norvegicus [Pathway:R-MMU-8986944] Transcriptional Regulation by MECP2 - Mus musculus
releaseDate	2018-09-12
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:9608119] Krishnaraj, Rahul, 2018-08-07 [InstanceEdit:9608114] Christodoulou, John, 2018-08-07
schemaClass	Pathway
species	[Species:48887] Homo sapiens
stId	R-HSA-8986944
summation	[Summation:9017788] MECP2 is an X chromosome gene whose loss-of-function mutatio...

Referrals

(hasEvent)	[Pathway:R-HSA-212436] Generic Transcription Pathway
(normalPathway)	[Pathway:R-HSA-9022534] Loss of MECP2 binding ability to 5hmC-DNA [Pathway:R-HSA-9022535] Loss of phosphorylation of MECP2 at T308 [Pathway:R-HSA-9022538] Loss of MECP2 binding ability to 5mC-DNA [Pathway:R-HSA-9022537] Loss of MECP2 binding ability to the NCoR/SMRT complex

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