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Merck
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安全性情報

WH0004204M1

Sigma-Aldrich

Monoclonal Anti-MECP2 antibody produced in mouse

clone 4B6, purified immunoglobulin, buffered aqueous solution

別名:

抗AUTSX3抗体, 抗DKFZp686A24160抗体, 抗MRX16抗体, 抗MRX79抗体, 抗RTS抗体

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About This Item

MDL番号:
UNSPSCコード:
12352203
NACRES:
NA.41

由来生物

mouse

品質水準

結合体

unconjugated

抗体製品の状態

purified immunoglobulin

抗体製品タイプ

primary antibodies

クローン

4B6, monoclonal

フォーム

buffered aqueous solution

化学種の反応性

mouse

テクニック

immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL

アイソタイプ

IgG2aκ

GenBankアクセッション番号

UniProtアクセッション番号

輸送温度

dry ice

保管温度

−20°C

ターゲットの翻訳後修飾

unmodified

遺伝子情報

human ... MECP2(4204)

詳細

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 most cases of Rett syndrome, a progressive neurologic developmental disorder and one of the most common causes of mental retardation in females. (provided by RefSeq)

免疫原

MECP2 (AAH11612, 81 a.a. ~ 170 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
PKQRRSIIRDRGPMYDDPTLPEGWTRKLKQRKSGRSAGKYDVYLINPQGKAFRSKVELIAYFEKVGDTSLDPNDFDFTVTGRGSPSRREQ

生物化学的/生理学的作用

Methyl-CpG binding protein 2 (MECP2) plays a vital role in regulation of a wide range of genes in the hypothalamus, and aids in both activation and repression of transcription. Mutation of the gene leads to a severe neurodevelopmental disorder associated with hypotonia, spasticity, severe learning disability, delayed psychomotor development, and recurrent pulmonary infections in males. Impairment in the expression of MeCP2 protein results in various neurological disorders such as Rett syndrome and Autism.

物理的形状

Solution in phosphate buffered saline, pH 7.4

法的情報

GenBank is a registered trademark of United States Department of Health and Human Services

免責事項

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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保管分類コード

10 - Combustible liquids

引火点(°F)

Not applicable

引火点(℃)

Not applicable

個人用保護具 (PPE)

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


適用法令

試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。

Jan Code

WH0004204M1-100UG:


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試験成績書(COA)

Lot/Batch Number

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文書ライブラリにアクセスする

Kamal K E Gadalla et al.
Molecular therapy. Methods & clinical development, 5, 180-190 (2017-05-13)
Rett syndrome (RTT), caused by loss-of-function mutations in the
Zhi Yi et al.
European journal of medical genetics, 59(6-7), 347-353 (2016-05-18)
Chromosome Xq28 duplications encompassing methyl-CpG-binding protein 2 gene (MECP2) are observed most in males with a severe neurodevelopmental disorder associated with hypotonia, spasticity, severe learning disability, delayed psychomotor development, and recurrent pulmonary infections. Most female carriers are asymptomatic due to
Maria Chahrour et al.
Science (New York, N.Y.), 320(5880), 1224-1229 (2008-05-31)
Mutations in the gene encoding the transcriptional repressor methyl-CpG binding protein 2 (MeCP2) cause the neurodevelopmental disorder Rett syndrome. Loss of function as well as increased dosage of the MECP2 gene cause a host of neuropsychiatric disorders. To explore the
X Nan et al.
Cell, 88(4), 471-481 (1997-02-21)
MeCP2 is an abundant mammalian protein that binds to methylated CpG. We have found that native and recombinant MeCP2 repress transcription in vitro from methylated promoters but do not repress nonmethylated promoters. Repression is nonlinearly dependent on the local density
Carlos Bueno et al.
PloS one, 11(4), e0153262-e0153262 (2016-04-12)
Dysfunctions of MeCP2 protein lead to various neurological disorders such as Rett syndrome and Autism. The exact functions of MeCP2 protein is still far from clear. At a molecular level, there exist contradictory data. MeCP2 protein is considered a single

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