Q9Z2D6 · MECP2_MOUSE
- ProteinMethyl-CpG-binding protein 2
- GeneMecp2
- StatusUniProtKB reviewed (Swiss-Prot)
- Organism
- Amino acids484 (go to sequence)
- Protein existenceEvidence at protein level
- Annotation score5/5
Function
function
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).
Features
Showing features for dna binding.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
DNA binding | 185-197 | A.T hook 1 | ||||
Sequence: GRGRGRPKGSGTG | ||||||
DNA binding | 265-277 | A.T hook 2 | ||||
Sequence: PKKRGRKPGSVVA |
GO annotations
all annotations | all molecular function | virus receptor activity | dna binding | rna binding | cytoskeletal motor activity | catalytic activity | gtpase activity | structural molecule activity | transporter activity | cytoskeletal protein binding | lipid binding | cyclase activity | antioxidant activity | oxidoreductase activity | transferase activity | hydrolase activity | lyase activity | isomerase activity | ligase activity | protein tag activity | cargo receptor activity | histone binding | protein folding chaperone | translation regulator activity | nutrient reservoir activity | receptor ligand activity | molecular transducer activity | molecular adaptor activity | toxin activity | cell adhesion mediator activity | molecular function regulator activity | virus coreceptor activity | catalytic activity, acting on a protein | catalytic activity, acting on dna | catalytic activity, acting on rna | molecular carrier activity | transcription regulator activity | general transcription initiation factor activity | molecular sensor activity | molecular sequestering activity | atp-dependent activity | other molecular function | all biological process | mitotic cell cycle | cytokinesis | cytoplasmic translation | immune system process | muscle system process | circulatory system process | renal system process | respiratory system process | carbohydrate metabolic process | generation of precursor metabolites and energy | dna replication | dna repair | dna recombination | chromatin organization | dna-templated transcription | regulation of dna-templated transcription | trna metabolic process | protein folding | protein glycosylation | amino acid metabolic process | modified amino acid metabolic process | lipid metabolic process | vitamin metabolic process | sulfur compound metabolic process | intracellular protein transport | nucleocytoplasmic transport | autophagy | inflammatory response | mitochondrion organization | cytoskeleton organization | microtubule-based movement | peroxisome organization | lysosome organization | chromosome segregation | cell adhesion | establishment or maintenance of cell polarity | programmed cell death | photosynthesis | mrna metabolic process | snrna metabolic process | vesicle-mediated transport | reproductive process | digestive system process | signaling | cell differentiation | protein catabolic process | extracellular matrix organization | regulatory ncrna-mediated gene silencing | telomere organization | cell junction organization | wound healing | ribosome biogenesis | cilium organization | anatomical structure development | cell motility | nervous system process | endocrine process | protein maturation | transmembrane transport | nucleobase-containing small molecule metabolic process | hepaticobiliary system process | membrane organization | protein-containing complex assembly | cell wall organization or biogenesis | nitrogen cycle metabolic process | protein localization to plasma membrane | defense response to other organism | detoxification | meiotic nuclear division | mitotic nuclear division | mitochondrial gene expression | carbohydrate derivative metabolic process | other biological process | all cellular component | nuclear chromosome | extracellular region | extracellular space | cell wall | nucleus | nuclear envelope | nucleoplasm | chromosome | nucleolus | mitochondrion | lysosome | endosome | vacuole | peroxisome | endoplasmic reticulum | golgi apparatus | lipid droplet | microtubule organizing center | cytosol | ribosome | cytoskeleton | plasma membrane | cilium | plastid | thylakoid | external encapsulating structure | extracellular matrix | cytoplasmic vesicle | organelle | other cellular component | |||
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Keywords
- Molecular function
- Biological process
Enzyme and pathway databases
Names & Taxonomy
Protein names
- Recommended nameMethyl-CpG-binding protein 2
- Short namesMeCp-2 protein; MeCp2
Gene names
Organism names
- Organism
- Strains
- Taxonomic lineageEukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus
Accessions
- Primary accessionQ9Z2D6
- Secondary accessions
Proteomes
Organism-specific databases
Subcellular Location
UniProt Annotation
GO Annotation
Note: Colocalized with methyl-CpG in the genome. Colocalized with TBL1X to the heterochromatin foci.
Keywords
- Cellular component
Phenotypes & Variants
Features
Showing features for mutagenesis.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Mutagenesis | 158 | Loss of localization to the nucleus. Distributes diffusely in cells. Interacts normally with NCOR2. | ||||
Sequence: T → M | ||||||
Mutagenesis | 302 | Abolishes interaction with NCOR2. | ||||
Sequence: P → R | ||||||
Mutagenesis | 304 | Abolishes interaction with NCOR2. | ||||
Sequence: K → E | ||||||
Mutagenesis | 305 | Abolishes interaction with NCOR2. | ||||
Sequence: K → R | ||||||
Mutagenesis | 306 | Abolishes interaction with NCOR2. Mice exhibit a severe neurological Rett syndrome-like phenotype. | ||||
Sequence: R → C |
Variants
We now provide the "Disease & Variants" viewer in its own tab.
The viewer provides 31 variants from UniProt as well as other sources including ClinVar and dbSNP.
PTM/Processing
Features
Showing features for chain, modified residue.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Chain | PRO_0000096347 | 1-484 | Methyl-CpG-binding protein 2 | |||
Sequence: MVAGMLGLREEKSEDQDLQGLRDKPLKFKKAKKDKKEDKEGKHEPLQPSAHHSAEPAEAGKAETSESSGSAPAVPEASASPKQRRSIIRDRGPMYDDPTLPEGWTRKLKQRKSGRSAGKYDVYLINPQGKAFRSKVELIAYFEKVGDTSLDPNDFDFTVTGRGSPSRREQKPPKKPKSPKAPGTGRGRGRPKGSGTGRPKAAASEGVQVKRVLEKSPGKLVVKMPFQASPGGKGEGGGATTSAQVMVIKRPGRKRKAEADPQAIPKKRGRKPGSVVAAAAAEAKKKAVKESSIRSVHETVLPIKKRKTRETVSIEVKEVVKPLLVSTLGEKSGKGLKTCKSPGRKSKESSPKGRSSSASSPPKKEHHHHHHHSESTKAPMPLLPSPPPPEPESSEDPISPPEPQDLSSSICKEEKMPRGGSLESDGCPKEPAKTQPMVATTTTVAEKYKHRGEGERKDIVSSSMPRPNREEPVDSRTPVTERVS | ||||||
Modified residue | 13 | Phosphoserine | ||||
Sequence: S | ||||||
Modified residue | 80 | Phosphoserine | ||||
Sequence: S | ||||||
Modified residue | 116 | Phosphoserine | ||||
Sequence: S | ||||||
Modified residue | 162 | Omega-N-methylarginine | ||||
Sequence: R | ||||||
Modified residue | 216 | Phosphoserine | ||||
Sequence: S | ||||||
Modified residue | 229 | Phosphoserine | ||||
Sequence: S | ||||||
Modified residue | 321 | N6-acetyllysine | ||||
Sequence: K | ||||||
Modified residue | 421 | Phosphoserine; by CaMK2 | ||||
Sequence: S | ||||||
Modified residue | 424 | Phosphoserine | ||||
Sequence: S | ||||||
Modified residue | 447 | N6-acetyllysine | ||||
Sequence: K |
Post-translational modification
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.
Keywords
- PTM
Proteomic databases
PTM databases
Expression
Gene expression databases
Interaction
Subunit
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; recruits TBL1X to the heterochromatin foci (By similarity).
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; recruits TBL1X to the heterochromatin foci (By similarity).
Binary interactions
Type | Entry 1 | Entry 2 | Number of experiments | Intact | |
---|---|---|---|---|---|
BINARY | Q9Z2D6 | Atrx Q61687 | 5 | EBI-1188816, EBI-2657527 | |
BINARY | Q9Z2D6 | Deaf1 Q9Z1T5 | 2 | EBI-1188816, EBI-2364863 | |
BINARY | Q9Z2D6 | Hdac3 O88895 | 5 | EBI-1188816, EBI-302263 | |
BINARY | Q9Z2D6 | Sin3a Q60520 | 5 | EBI-1188816, EBI-349034 | |
BINARY | Q9Z2D6 | Smarca2 O35846 | 2 | EBI-1188816, EBI-371564 | |
BINARY | Q9Z2D6 | Smarca4 Q3TKT4 | 2 | EBI-1188816, EBI-1210244 | |
BINARY | Q9Z2D6 | Smc1a Q9CU62 | 3 | EBI-1188816, EBI-2550016 | |
BINARY | Q9Z2D6 | Smc3 Q9CW03 | 3 | EBI-1188816, EBI-2550068 | |
BINARY | Q9Z2D6 | Sp3 O70494 | 2 | EBI-1188816, EBI-643514 | |
BINARY | Q9Z2D6-1 | Foxg1 Q60987 | 4 | EBI-26609102, EBI-11166131 | |
BINARY | Q9Z2D6-2 | Foxg1 Q60987 | 2 | EBI-26609115, EBI-11166131 |
Protein-protein interaction databases
Miscellaneous
Structure
Family & Domains
Features
Showing features for region, compositional bias, domain.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Region | 1-119 | Disordered | ||||
Sequence: MVAGMLGLREEKSEDQDLQGLRDKPLKFKKAKKDKKEDKEGKHEPLQPSAHHSAEPAEAGKAETSESSGSAPAVPEASASPKQRRSIIRDRGPMYDDPTLPEGWTRKLKQRKSGRSAGK | ||||||
Compositional bias | 8-49 | Basic and acidic residues | ||||
Sequence: LREEKSEDQDLQGLRDKPLKFKKAKKDKKEDKEGKHEPLQPS | ||||||
Compositional bias | 83-109 | Basic and acidic residues | ||||
Sequence: QRRSIIRDRGPMYDDPTLPEGWTRKLK | ||||||
Domain | 90-162 | MBD | ||||
Sequence: DRGPMYDDPTLPEGWTRKLKQRKSGRSAGKYDVYLINPQGKAFRSKVELIAYFEKVGDTSLDPNDFDFTVTGR | ||||||
Region | 147-216 | Disordered | ||||
Sequence: DTSLDPNDFDFTVTGRGSPSRREQKPPKKPKSPKAPGTGRGRGRPKGSGTGRPKAAASEGVQVKRVLEKS | ||||||
Region | 249-278 | Disordered | ||||
Sequence: KRPGRKRKAEADPQAIPKKRGRKPGSVVAA | ||||||
Compositional bias | 251-267 | Basic and acidic residues | ||||
Sequence: PGRKRKAEADPQAIPKK | ||||||
Region | 269-309 | Interaction with NCOR2 | ||||
Sequence: GRKPGSVVAAAAAEAKKKAVKESSIRSVHETVLPIKKRKTR | ||||||
Region | 285-309 | Interaction with TBL1XR1 | ||||
Sequence: KKAVKESSIRSVHETVLPIKKRKTR | ||||||
Region | 319-484 | Disordered | ||||
Sequence: VVKPLLVSTLGEKSGKGLKTCKSPGRKSKESSPKGRSSSASSPPKKEHHHHHHHSESTKAPMPLLPSPPPPEPESSEDPISPPEPQDLSSSICKEEKMPRGGSLESDGCPKEPAKTQPMVATTTTVAEKYKHRGEGERKDIVSSSMPRPNREEPVDSRTPVTERVS | ||||||
Compositional bias | 380-398 | Pro residues | ||||
Sequence: MPLLPSPPPPEPESSEDPI | ||||||
Compositional bias | 446-484 | Basic and acidic residues | ||||
Sequence: EKYKHRGEGERKDIVSSSMPRPNREEPVDSRTPVTERVS |
Keywords
- Domain
Phylogenomic databases
Family and domain databases
Sequence & Isoform
- Sequence statusComplete
This entry describes 2 isoforms produced by Alternative splicing.
Q9Z2D6-1
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.
- NameA
- SynonymsBeta
- Length484
- Mass (Da)52,307
- Last updated1999-05-01 v1
- Checksum62FD228F0118A49F
Q9Z2D6-2
- NameB
- SynonymsAlpha
- NoteTen times higher expression levels than isoform A in brain (at protein level).
- Differences from canonical
- 1-9: MVAGMLGLR → MAAAAATAAAAAAPSGGGGGGEEERL
Computationally mapped potential isoform sequences
There are 2 potential isoforms mapped to this entry
Features
Showing features for alternative sequence, compositional bias, sequence conflict.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Alternative sequence | VSP_022949 | 1-9 | in isoform B | |||
Sequence: MVAGMLGLR → MAAAAATAAAAAAPSGGGGGGEEERL | ||||||
Compositional bias | 8-49 | Basic and acidic residues | ||||
Sequence: LREEKSEDQDLQGLRDKPLKFKKAKKDKKEDKEGKHEPLQPS | ||||||
Compositional bias | 83-109 | Basic and acidic residues | ||||
Sequence: QRRSIIRDRGPMYDDPTLPEGWTRKLK | ||||||
Compositional bias | 251-267 | Basic and acidic residues | ||||
Sequence: PGRKRKAEADPQAIPKK | ||||||
Sequence conflict | 328 | in Ref. 4; BAE34602 | ||||
Sequence: L → P | ||||||
Compositional bias | 380-398 | Pro residues | ||||
Sequence: MPLLPSPPPPEPESSEDPI | ||||||
Compositional bias | 446-484 | Basic and acidic residues | ||||
Sequence: EKYKHRGEGERKDIVSSSMPRPNREEPVDSRTPVTERVS |
Keywords
- Coding sequence diversity
- Technical term
Sequence databases
Nucleotide Sequence | Protein Sequence | Molecule Type | Status | |
---|---|---|---|---|
AF072251 EMBL· GenBank· DDBJ | AAC68880.1 EMBL· GenBank· DDBJ | mRNA | ||
AJ132922 EMBL· GenBank· DDBJ | CAB46495.1 EMBL· GenBank· DDBJ | mRNA | ||
AF121351 EMBL· GenBank· DDBJ | - | Genomic DNA | No translation available. | |
AF158181 EMBL· GenBank· DDBJ | AAF33024.1 EMBL· GenBank· DDBJ | mRNA | ||
AK158664 EMBL· GenBank· DDBJ | BAE34602.1 EMBL· GenBank· DDBJ | mRNA | ||
AL672002 EMBL· GenBank· DDBJ | - | Genomic DNA | No translation available. | |
BC027153 EMBL· GenBank· DDBJ | AAH27153.1 EMBL· GenBank· DDBJ | mRNA |