Methyl CGp-binding protein 2

Catalog#	Product Description	Host Species	Nature	Cross reactivity	Quantity	Volume	Price
MeCP2-201AP	MeCP2 antibodies	Rabbit	Affinity Purified	r, m, h	100.0 ug	150-500 ul	$245.00
FITC-MeCP2	FITC-conjugated MeCP2 antibodies	Rabbit	FITC-conjugated antibodies	r, m, h	100.0 ug	150-500 ul	$350.00
P-MeCP2	Antigenic blocking peptide	n/a	Synthetic peptide	n/a	250.0 ug	100 ul	$125.00
PC-MeCP2	WB positive control for MeCP2 antibodies	n/a	Protein in ready-to-use buffer	n/a	0.0 ug	150 ul	$185.00

The epigenetic information encoded as methylated CpG dinucleotides to the transcriptional machinery is transferred by a multifunction methylated DNA binding protein called Methylated CpG biding protein 2. Mutation in MeCP2 protein led to debilitating neurogenerative X-linked autism-spectrum neurodevelopmental disease Rhett syndrome (RTT). Several functional domains have been identified on the MeCP2 protein including Methylated non-methylated DNA binding domains in the first 294 amino acids and a chromatin binding motif near the C-terminal DNA region of the protein (1). The genetic and epigenetic defects led to reduced expression of MeCP2 protein in brain of was autism patients, it may be an important factor in the complex etiology of this disease (2). The gene is located on Xq28 chromosome. Defects in the MeCP2 gene care caused by Angelman syndrome also known as happy puppet syndrome which a neuron-developmental disorder characterized by severe mental retardation, absent of speech, ataxia, sociable affects and dysmorphic facial features similar to Rhett syndrome clinical feature. Rett syndrome (RS) is an X-linked neuron-developmental disorder and the second most common cause of genetic mental retardation in females. Different mutations in MECP2 are found in up to 95% of typical cases of RS. The onset of this disease is caused by non progressive encephalopathy with neonatal onset. The defects in MeCP2 is cause of X-linked mental retardation, Autism 3 (AUTSX3) about 80% of the Rhett syndrome cases have mutational defects in the MeCP2 gene. Expression of this protein sprout generation of neuron and reversal of neurological defects in a moue model of Rett syndrome (3).

MeCP2 protein has 2 AT hook DNA biding domains, a transcriptional repression domain (TRD) and one MBD or Methyl CpG binding domain on the amino terminal end (1-96). Methyl CGp binding protein 2 gene is located on fragile X chromosome and this chromosomal protein binds to methylated DNA. It can bind specifically to a single methyl-CpG pair. The binding is not influenced by sequences flanking the methyl-CpGs. Upon binding it mediates transcriptional repression through interaction with histone deacetylase and co repressor SIN3A. MeCP2, is responsible for transcriptional repression of the leukosialin gene by repression of the Sp1-activated trasnacription. Methyl-CpG-binding protein MeCP2 represses Sp1-activated transcription of the human leukosialin gene when the promoter is methylated (3).

The MeCP2-selective antibodies were generated against unique antigenic sequences form MeCP2 protein that are not found in any other protein in the gene bank. The MeCP2 antibodies were affinity purified over immobilized antigen based chromatography, and the purified immunoglobulins are stabilized in antibody stabilization buffer. FabGennix Int. Inc., will also provide limited quantities of antigenic blocking peptide for MeCP-201AP. Antibodies to several other neuro-developmental disorder are also available from FabGennix International Inc. For a complete list of antibodies please visit www.FabGennix.com. FabGennix Inc. will conjugate antibodies with secondary enzymes (alk-Pase or HRP) or fluorescent probes upon request at a nominal cost. FabGennix Int. Inc., will also provide western blot positive controls for it antibodies in ready-to-use buffer.

For research use only, not for diagnostic or therapeutic use.

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Methyl CGp-binding protein 2

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