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SRP0324

Sigma-Aldrich

MECP2 human

recombinant, expressed in E. coli, ≥70% (SDS-PAGE)

Synonym(s):

MRX16, PPMX, RTS, methyl CpG binding protein 2 (Rett syndrome)

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About This Item

UNSPSC Code:
12352202
NACRES:
NA.32

biological source

human

recombinant

expressed in E. coli

Assay

≥70% (SDS-PAGE)

form

aqueous solution

mol wt

36 kDa

packaging

pkg of 50 μg

NCBI accession no.

UniProt accession no.

shipped in

dry ice

storage temp.

−70°C

Gene Information

human ... MECP2(4204)

General description

Human recombinant Methyl CpG binding Protein 2 (Rett syndrome) (MECP2), (GenBank Accession No. NM_004992), amino acids 78-162 with N-terminal GST-tag, MW= 36 kDa, expressed in an E. coli expression system.
Methyl-CpG binding protein 2 (MECP2) is encoded by the gene mapped to human chromosome Xq28. Higher molecular weight form of hMeCP2 is expressed in frontal cortex nuclear and synaptic fractions, and in lymphoid cells. Fibroblast and lymphoblastoid strains of female patients with Rett syndrome and MeCP2 transfected cells also show expression of high molecular weight form of MeCP2.2The encoded chromosomal protein is characterized with the 80 amino acid-containing methyl-CpG binding domain, involved in chromosomal localization of the protein.

Application

Useful for the study of enzyme kinetics, screening inhibitors, and selectivity profiling.

Biochem/physiol Actions

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.

Storage Class Code

12 - Non Combustible Liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


Certificates of Analysis (COA)

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Yu-Chiao Huang et al.
Plant molecular biology, 90(1-2), 127-135 (2015-11-02)
Sucrose synthase (SuS), which catalyzes the reversible conversion of sucrose and uridine diphosphate (UDP) into fructose and UDP-glucose, is a key enzyme in sucrose metabolism in higher plants. SuS belongs to family 4 of the glycosyltransferases (GT4) and contains an
MeCP2 is a transcriptional repressor with abundant binding sites in genomic chromatin.
Nan X
Cell, 88, 471-481 (1997)
Rett Syndrome Mutant Neural Cells Lacks MeCP2 Immunoreactive Bands.
Bueno C
PLoS ONE, 11 (2016)
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
R E Amir et al.
Nature genetics, 23(2), 185-188 (1999-10-03)
Rett syndrome (RTT, MIM 312750) is a progressive neurodevelopmental disorder and one of the most common causes of mental retardation in females, with an incidence of 1 in 10,000-15,000 (ref. 2). Patients with classic RTT appear to develop normally until

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