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| Description | |
|---|---|
| Catalogue Number | 07-013-25UG |
| Brand Family | Upstate |
| Trade Name |
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| Description | Anti-MeCP2 |
| Alternate Names |
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| Background Information | NA methyltransferases methylate the 5-position of cytosine in the context of CpG dinucleotides. DNA methylation is crucial for normal embryonic development, imprinting, and X chromosome inactivation. Methyl CpG binding proteins (MeCPs) specifically recognize methylated regions of DNA and repress transcription both directly and by association with known corepressor proteins which include members of the histone deacetylase protein families. |
| Product Information | |
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| Format | Purified |
| Control |
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| Presentation | Purified rabbit polyclonal IgG in buffer containing 0.1 M Tris-glycine, pH 7.4, 0.15 M NaCl, 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
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| Application | Use Anti-MeCP2 Antibody (rabbit polyclonal antibody) validated in WB to detect MeCP2 also known as MeCP-2 protein, mental retardation X-linked 16, mental retardation X-linked 79, methyl CpG binding protein 2. |
| Key Applications |
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| Biological Information | |
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| Immunogen | KLH-conjugated, synthetic peptide corresponding to amino acids 465-478 of mouse MeCP2 (C-PRPNREEPVDSRTP) with an N-terminal cysteine added for conjugation purposes. |
| Concentration | Please refer to lot specific datasheet. |
| Host | Rabbit |
| Specificity | Recognizes MeCP2, Mr 75 kDa. A protein at ~75 kDa was also observed in HeLa nuclear extract. This protein was competed by pre-incubation of the antibody with the immunogenic peptide, suggesting that some HeLa strains contain MeCP2 or a related protein, contrary to a previous report. |
| Isotype | IgG |
| Species Reactivity |
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| Species Reactivity Note | Mouse and rat. Predicted to cross-react with human based on the conservation of the immunogenic sequence. |
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
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| Entrez Gene Summary | DNA methylation is the major modification of eukaryotic genomes and plays an essential role in mammalian development. Human proteins MECP2, MBD1, MBD2, MBD3, and MBD4 comprise a family of nuclear proteins related by the presence in each of a methyl-CpG binding domain (MBD). Each of these proteins, with the exception of MBD3, is capable of binding specifically to methylated DNA. MECP2, MBD1 and MBD2 can also repress transcription from methylated gene promoters. In contrast to other MBD family members, MECP2 is X-linked and subject to X inactivation. MECP2 is dispensible in stem cells, but is essential for embryonic development. MECP2 gene mutations are the cause of some cases of Rett syndrome, a progressive neurologic developmental disorder and one of the most common causes of mental retardation in females. |
| Gene Symbol |
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| Purification Method | Protein A Purfied |
| UniProt Number |
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| UniProt Summary | FUNCTION: SwissProt: P51608 # 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 SIN3A. SIZE: 486 amino acids; 52441 Da SUBUNIT: Interacts with FNBP3 (By similarity). SUBCELLULAR LOCATION: Nucleus. Note=Colocalized with methyl-CpG in the genome. TISSUE SPECIFICITY: Present in all adult somatic tissues tested. PTM: Phosphorylated on Ser-423 in brain upon synaptic activity, which attenuates its repressor activity and seems to regulate dendritic growth and spine maturation (By similarity). DISEASE: SwissProt: P51608 # Defects in MECP2 may be a cause of Angelman syndrome (AS) [MIM:105830]; also known as happy puppet syndrome. AS is a neurodevelopmental disorder characterized by severe mental retardation, absent speech, ataxia, sociable affect and dysmorphic facial features. AS and Rett syndrome have overlapping clinical features. & Defects in MECP2 are the cause of mental retardation syndromic X-linked type 13 (MRXS13) [MIM:300055]. Mental retardation is a mental disorder characterized by significantly sub-average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. MRXS13 patients manifest mental retardation associated with other variable features such as spasticity, episodes of manic depressive psychosis, increased tone and macroorchidism. & Defects in MECP2 are the cause of Rett syndrome (RTT) [MIM:312750]. RTT is an X-linked dominant disease, it is a progressive neurologic developmental disorder and one of the most common causes of mental retardation in females. Patients appear to develop normally until 6 to 18 months of age, then gradually lose speech and purposeful hand movements and develop microcephaly, seizures, autism, ataxia, intermittent hyperventilation, and stereotypic hand movements. After initial regression, the condition stabilizes and patients usually survive into adulthood. & Defects in MECP2 may be the cause of susceptibility to X- linked autism 3 (AUTSX3) [MIM:300496]. AUTSX3 is a pervasive developmental disorder (PDD), prototypically characterized by impairments in reciprocal social interaction and communication, restricted and stereotyped patterns of interests and activities, and the presence of developmental abnormalities by 3 years of age. & Defects in MECP2 may be a cause of nonprogressive encephalopathy with neonatal onset [MIM:300005]. & A chromosomal duplication involving MECP2 is the cause of mental retardation syndromic X-linked Lubs type (MRXSL) [MIM:300260]. Increased dosage of MECP2 appears to be responsible for the mental retardation phenotype. The main features present in affected males are severe to profound mental retardation with onset at birth, axial and facial hypotonia, progressive spasticity predominantly at the lower limbs, seizures and recurrent infections. SIMILARITY: SwissProt: P51608 ## Contains 2 A.T hook DNA-binding domains. & Contains 1 MBD (methyl-CpG-binding) domain. |
| Molecular Weight | ~75 kDa observed |
| Product Usage Statements | |
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| Quality Assurance | Evaluated by Western Blot on Mouse Brain lysates. |
| Usage Statement |
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| Storage and Shipping Information | |
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| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
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| Material Size | 25 μg |