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Protein

Methyl-CpG-binding protein 2

Gene

Mecp2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Chromosomal protein that binds to methylated DNA. It can bind specifically to a single methyl-CpG pair. It is not influenced by sequences flanking the methyl-CpGs. Mediates transcriptional repression through interaction with histone deacetylase and the corepressor SIN3. Binds both 5-methylcytosine (5mC) and 5-hydroxymethylcytosine (5hmC)-containing DNA, with a preference for 5-methylcytosine (5mC).1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
DNA bindingi185 – 197A.T hook 1Add BLAST13
DNA bindingi265 – 277A.T hook 2Add BLAST13

GO - Molecular functioni

  • chromatin binding Source: MGI
  • chromatin DNA binding Source: MGI
  • DNA binding Source: MGI
  • DNA binding transcription factor activity Source: MGI
  • double-stranded methylated DNA binding Source: MGI
  • enzyme binding Source: MGI
  • four-way junction DNA binding Source: MGI
  • histone deacetylase binding Source: MGI
  • methyl-CpG binding Source: MGI
  • mRNA binding Source: MGI
  • promoter-specific chromatin binding Source: MGI
  • protein domain specific binding Source: MGI
  • protein N-terminus binding Source: MGI
  • siRNA binding Source: MGI
  • transcriptional repressor activity, RNA polymerase II proximal promoter sequence-specific DNA binding Source: BHF-UCL
  • transcription factor binding Source: MGI
  • unmethylated CpG binding Source: MGI

GO - Biological processi

  • adult locomotory behavior Source: MGI
  • behavioral fear response Source: MGI
  • brain development Source: MGI
  • cardiolipin metabolic process Source: MGI
  • catecholamine secretion Source: MGI
  • cellular biogenic amine metabolic process Source: MGI
  • cellular response to isoquinoline alkaloid Source: MGI
  • cellular response to potassium ion Source: MGI
  • cerebellum development Source: MGI
  • chemical synaptic transmission Source: MGI
  • chromatin silencing Source: MGI
  • dendrite development Source: MGI
  • excitatory postsynaptic potential Source: MGI
  • glucocorticoid metabolic process Source: MGI
  • glutamine metabolic process Source: MGI
  • histone acetylation Source: MGI
  • histone methylation Source: MGI
  • inositol metabolic process Source: MGI
  • learning Source: MGI
  • long-term memory Source: MGI
  • long-term synaptic potentiation Source: MGI
  • memory Source: MGI
  • mitochondrial electron transport, ubiquinol to cytochrome c Source: MGI
  • mitotic spindle organization Source: MGI
  • multicellular organismal response to stress Source: MGI
  • negative regulation of angiogenesis Source: MGI
  • negative regulation of astrocyte differentiation Source: MGI
  • negative regulation of blood vessel endothelial cell migration Source: MGI
  • negative regulation of dendrite extension Source: MGI
  • negative regulation of dendritic spine development Source: MGI
  • negative regulation of gene expression Source: MGI
  • negative regulation of histone acetylation Source: MGI
  • negative regulation of histone methylation Source: MGI
  • negative regulation of neuron apoptotic process Source: MGI
  • negative regulation of primary miRNA processing Source: MGI
  • negative regulation of protein binding Source: MGI
  • negative regulation of smooth muscle cell differentiation Source: BHF-UCL
  • negative regulation of transcription, DNA-templated Source: MGI
  • negative regulation of transcription by RNA polymerase II Source: MGI
  • negative regulation of transcription from RNA polymerase II promoter involved in smooth muscle cell differentiation Source: BHF-UCL
  • neurological system process involved in regulation of systemic arterial blood pressure Source: MGI
  • neuromuscular process Source: MGI
  • neuromuscular process controlling posture Source: MGI
  • neuron differentiation Source: MGI
  • neuron maturation Source: MGI
  • neuron projection development Source: MGI
  • pathogenesis Source: MGI
  • phosphatidylcholine metabolic process Source: MGI
  • positive regulation of anterograde dense core granule transport Source: MGI
  • positive regulation of branching morphogenesis of a nerve Source: MGI
  • positive regulation of cell proliferation Source: MGI
  • positive regulation of dendrite extension Source: MGI
  • positive regulation of dendritic spine development Source: MGI
  • positive regulation of DNA methylation Source: MGI
  • positive regulation of G2/M transition of mitotic cell cycle Source: MGI
  • positive regulation of gene expression Source: MGI
  • positive regulation of histone H3-K9 trimethylation Source: BHF-UCL
  • positive regulation of microtubule nucleation Source: MGI
  • positive regulation of retrograde dense core granule transport Source: MGI
  • positive regulation of synapse assembly Source: MGI
  • positive regulation of synaptic plasticity Source: MGI
  • positive regulation of transcription, DNA-templated Source: MGI
  • post-embryonic development Source: MGI
  • proprioception Source: MGI
  • protein localization Source: MGI
  • regulation of DNA methylation Source: MGI
  • regulation of gene expression Source: MGI
  • regulation of gene expression, epigenetic Source: MGI
  • regulation of gene expression by genetic imprinting Source: MGI
  • regulation of respiratory gaseous exchange by neurological system process Source: MGI
  • regulation of synaptic plasticity Source: MGI
  • regulation of transcription, DNA-templated Source: MGI
  • respiratory gaseous exchange Source: MGI
  • response to cocaine Source: MGI
  • response to hypoxia Source: MGI
  • sensory perception of pain Source: MGI
  • social behavior Source: MGI
  • startle response Source: MGI
  • synapse assembly Source: MGI
  • transcription, DNA-templated Source: UniProtKB-KW
  • ventricular system development Source: MGI
  • visual learning Source: MGI

Keywordsi

Molecular functionDNA-binding, Repressor
Biological processTranscription, Transcription regulation

Names & Taxonomyi

Protein namesi
Recommended name:
Methyl-CpG-binding protein 2
Short name:
MeCp-2 protein
Short name:
MeCp2
Gene namesi
Name:Mecp2
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome X

Organism-specific databases

MGIiMGI:99918 Mecp2

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi158T → M: Loss of localization to the nucleus. Distributes diffusely in cells. Interacts normally with NCOR2. 1 Publication1
Mutagenesisi302P → R: Abolishes interaction with NCOR2. 1 Publication1
Mutagenesisi304K → E: Abolishes interaction with NCOR2. 1 Publication1
Mutagenesisi305K → R: Abolishes interaction with NCOR2. 1 Publication1
Mutagenesisi306R → C: Abolishes interaction with NCOR2. Mice exhibit a severe neurological Rett syndrome-like phenotype. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000963471 – 484Methyl-CpG-binding protein 2Add BLAST484

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei13PhosphoserineBy similarity1
Modified residuei80PhosphoserineCombined sources1
Modified residuei116PhosphoserineBy similarity1
Modified residuei162Omega-N-methylarginineCombined sources1
Modified residuei216PhosphoserineBy similarity1
Modified residuei229PhosphoserineCombined sources1
Modified residuei321N6-acetyllysineCombined sources1
Modified residuei421Phosphoserine; by CaMK21 Publication1
Modified residuei424PhosphoserineCombined sources1
Modified residuei447N6-acetyllysineCombined sources1

Post-translational modificationi

Phosphorylated on Ser-421 by CaMK2 in brain upon synaptic activity, which attenuates its repressor activity and seems to regulate dendritic growth and spine maturation. Does not seem to be phosphorylated on Ser-421 in other tissues.1 Publication

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiQ9Z2D6
PaxDbiQ9Z2D6
PeptideAtlasiQ9Z2D6
PRIDEiQ9Z2D6

PTM databases

iPTMnetiQ9Z2D6
PhosphoSitePlusiQ9Z2D6

Expressioni

Gene expression databases

BgeeiENSMUSG00000031393
CleanExiMM_MECP2
ExpressionAtlasiQ9Z2D6 baseline and differential
GenevisibleiQ9Z2D6 MM

Interactioni

Subunit structurei

Interacts with FNBP3 (PubMed:9171351). Interacts with CDKL5 (By similarity). Interacts with ATRX; MECP2 recruits ATRX to pericentric heterochromatin in neuronal cells (PubMed:17296936). Interacts with NCOR2 (PubMed:23770565). Interacts with TBL1XR1; bridges interaction between MECP2 and NCOR1 (PubMed:28348241). Interacts with TBL1X; recuits TBL1X to the heterochromatin foci (By similarity).By similarity4 Publications

Binary interactionsi

Show more details

GO - Molecular functioni

Protein-protein interaction databases

BioGridi201380, 10 interactors
CORUMiQ9Z2D6
DIPiDIP-39984N
IntActiQ9Z2D6, 12 interactors
MINTiQ9Z2D6
STRINGi10090.ENSMUSP00000033770

Structurei

Secondary structure

1484
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi298 – 300Combined sources3
Helixi303 – 305Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5NAFX-ray2.49E/F285-309[»]
ProteinModelPortaliQ9Z2D6
SMRiQ9Z2D6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini90 – 162MBDPROSITE-ProRule annotationAdd BLAST73

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni269 – 309Interaction with NCOR21 PublicationAdd BLAST41
Regioni285 – 309Interaction with TBL1XR11 PublicationAdd BLAST25

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi366 – 372His-rich7
Compositional biasi379 – 403Pro-richAdd BLAST25

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiKOG4161 Eukaryota
ENOG41126JX LUCA
GeneTreeiENSGT00530000063687
HOGENOMiHOG000015809
HOVERGENiHBG052445
InParanoidiQ9Z2D6
KOiK11588
OMAiKMPRAGS
OrthoDBiEOG091G05HO
PhylomeDBiQ9Z2D6
TreeFamiTF332974

Family and domain databases

InterProiView protein in InterPro
IPR016177 DNA-bd_dom_sf
IPR017353 Me_CpG-bd_MeCP2
IPR001739 Methyl_CpG_DNA-bd
PANTHERiPTHR15074:SF4 PTHR15074:SF4, 1 hit
PfamiView protein in Pfam
PF01429 MBD, 1 hit
PIRSFiPIRSF038006 Methyl_CpG_bd_MeCP2, 1 hit
SMARTiView protein in SMART
SM00391 MBD, 1 hit
SUPFAMiSSF54171 SSF54171, 1 hit
PROSITEiView protein in PROSITE
PS50982 MBD, 1 hit

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform A (identifier: Q9Z2D6-1) [UniParc]FASTAAdd to basket
Also known as: Beta

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MVAGMLGLRE EKSEDQDLQG LRDKPLKFKK AKKDKKEDKE GKHEPLQPSA
60 70 80 90 100
HHSAEPAEAG KAETSESSGS APAVPEASAS PKQRRSIIRD RGPMYDDPTL
110 120 130 140 150
PEGWTRKLKQ RKSGRSAGKY DVYLINPQGK AFRSKVELIA YFEKVGDTSL
160 170 180 190 200
DPNDFDFTVT GRGSPSRREQ KPPKKPKSPK APGTGRGRGR PKGSGTGRPK
210 220 230 240 250
AAASEGVQVK RVLEKSPGKL VVKMPFQASP GGKGEGGGAT TSAQVMVIKR
260 270 280 290 300
PGRKRKAEAD PQAIPKKRGR KPGSVVAAAA AEAKKKAVKE SSIRSVHETV
310 320 330 340 350
LPIKKRKTRE TVSIEVKEVV KPLLVSTLGE KSGKGLKTCK SPGRKSKESS
360 370 380 390 400
PKGRSSSASS PPKKEHHHHH HHSESTKAPM PLLPSPPPPE PESSEDPISP
410 420 430 440 450
PEPQDLSSSI CKEEKMPRGG SLESDGCPKE PAKTQPMVAT TTTVAEKYKH
460 470 480
RGEGERKDIV SSSMPRPNRE EPVDSRTPVT ERVS
Length:484
Mass (Da):52,307
Last modified:May 1, 1999 - v1
Checksum:i62FD228F0118A49F
GO
Isoform B (identifier: Q9Z2D6-2) [UniParc]FASTAAdd to basket
Also known as: Alpha

The sequence of this isoform differs from the canonical sequence as follows:
     1-9: MVAGMLGLR → MAAAAATAAAAAAPSGGGGGGEEERL

Note: Ten times higher expression levels than isoform A in brain (at protein level).
Show »
Length:501
Mass (Da):53,576
Checksum:iE88FC12C21F95F4C
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti328L → P in BAE34602 (PubMed:16141072).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0229491 – 9MVAGMLGLR → MAAAAATAAAAAAPSGGGGG GEEERL in isoform B. 1 Publication9

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF072251 mRNA Translation: AAC68880.1
AJ132922 mRNA Translation: CAB46495.1
AF121351 Genomic DNA No translation available.
AF158181 mRNA Translation: AAF33024.1
AK158664 mRNA Translation: BAE34602.1
AL672002 Genomic DNA Translation: CAM18741.1
AL672002 Genomic DNA Translation: CAM18742.1
BC027153 mRNA Translation: AAH27153.1
CCDSiCCDS41016.1 [Q9Z2D6-2]
CCDS41017.1 [Q9Z2D6-1]
RefSeqiNP_001075448.1, NM_001081979.2 [Q9Z2D6-2]
NP_034918.1, NM_010788.4 [Q9Z2D6-1]
UniGeneiMm.131408
Mm.414303

Genome annotation databases

EnsembliENSMUST00000033770; ENSMUSP00000033770; ENSMUSG00000031393 [Q9Z2D6-2]
ENSMUST00000100750; ENSMUSP00000098314; ENSMUSG00000031393 [Q9Z2D6-1]
ENSMUST00000170481; ENSMUSP00000127115; ENSMUSG00000031393 [Q9Z2D6-1]
GeneIDi17257
KEGGimmu:17257
UCSCiuc009tnt.3 mouse [Q9Z2D6-1]
uc009tnu.3 mouse [Q9Z2D6-2]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiMECP2_MOUSE
AccessioniPrimary (citable) accession number: Q9Z2D6
Secondary accession number(s): B1AUZ2, B1AUZ3, Q3TYG1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 24, 2001
Last sequence update: May 1, 1999
Last modified: June 20, 2018
This is version 175 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

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