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Human AMT Recombinant Protein (RPPB1401)

SKU:
RPPB1401
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P48728
Research Area:
Enzymes
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Description

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Product Name:Human AMT Recombinant Protein
Product Code:RPPB1401
Size:20µg
Species:Human
Target:AMT
Synonyms:GCE, GCST, GCVT, NKH, Aminomethyltransferase,mitochondrial, Glycine cleavage system T protein, GCVT.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:AMT protein solution (1mg/ml) in Phosphate Buffered Saline, 30% glycerol and 1mM DTT.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSAQEVLRR TPLYDFHLAH GGKMVAFAGW SLPVQYRDSH TDSHLHTRQHCSLFDVSHML QTKILGSDRV KLMESLVVGD IAELRPNQGT LSLFTNEAGG ILDDLIVTNT SEGHLYVVSNAGCWEKDLAL MQDKVRELQN QGRDVGLEVL DNALLALQGP TAAQVLQAGV ADDLRKLPFM TSAVMEVFGVSGCRVTRCGY TGEDGVEISV PVAGAVHLAT AILKNPEVKL AGLAARDSLR LEAGLCLYGN DIDEHTTPVEGSLSWTLGKR RRAAMDFPGA KVIVPQLKGR VQRRRVGLMC EGAPMRAHSP ILNMEGTKIG TVTSGCPSPSLKKNVAMGYV PCEYSRPGTM LLVEVRRKQQ MAVVSKMPFV PTNYYTLK

Aminomethyltransferase, mitochondrial isoform 1 (AMT) is a component of the glycine cleavage system termed T-protein. AMT reversibly catalyzes the degradation of the aminomethyl moiety of glycine attached to the lipoate cofactor of H-protein, leading to the production of ammonia, 5,10-methylenetetrahydrofolate, and dihydrolipoate-bearing H-protein in the presence of tetrahydrofolate.

AMT Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 398 amino acids (29-403 a.a) and having a molecular mass of 43.3kDa.AMT is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function:AMT: The glycine cleavage system catalyzes the degradation of glycine. Defects in AMT are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvT family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Amino Acid Metabolism - glycine, serine and threonine; Energy Metabolism - nitrogen; Methyltransferase; Mitochondrial; EC 2.1.2.10; Cofactor and Vitamin Metabolism - one carbon pool by folate

Chromosomal Location of Human Ortholog: 3p21.31

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function:aminomethyltransferase activity

Biological Process: glycine catabolic process; glycine decarboxylation via glycine cleavage system; glyoxylate metabolic process

Disease: Glycine Encephalopathy

NCBI Summary:This gene encodes one of four critical components of the glycine cleavage system. Mutations in this gene have been associated with glycine encephalopathy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2011]
UniProt Code:P48728
NCBI GenInfo Identifier:1346122
NCBI Gene ID:275
NCBI Accession:P48728.1
UniProt Secondary Accession:P48728,Q96IG6, A8K3I5, B4DE61, B4DJQ0, E9PBG1,
UniProt Related Accession:P48728
Molecular Weight:41,780 Da
NCBI Full Name:Aminomethyltransferase, mitochondrial
NCBI Synonym Full Names:aminomethyltransferase
NCBI Official Symbol:AMT��
NCBI Official Synonym Symbols:GCE; NKH; GCST; GCVT��
NCBI Protein Information:aminomethyltransferase, mitochondrial
UniProt Protein Name:Aminomethyltransferase, mitochondrial
UniProt Synonym Protein Names:Glycine cleavage system T protein
Protein Family:Probable ammonia channel
UniProt Gene Name:AMT��
UniProt Entry Name:GCST_HUMAN

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