Recombinant Human Guanidinoacetate N-Methyltransferase/GAMT

Instruction Manual!

Product name:	Recombinant Human Guanidinoacetate N-Methyltransferase/GAMT
Source:	E. coli
Purity:	Greater than 95% as determined by reducing SDS-PAGE.
Buffer Formulation:	Supplied as a 0.2 μm filtered solution of 20mM TrisHCl,1mMDTT,pH 8.0 .
Applications:	Applications:SDS-PAGE; WB; ELISA; IP.
Storage:	Avoid repeated freeze/thaw cycles. Store at 2-8 oC for one month. Aliquot and store at -80 oC for 12 months.
UOM:	100ug/50ug/200ug/1mg/1g

Source	E. coli
Description	Recombinant Human GAMT is produced by our E.coli expression system and the target gene encoding Met1-Gly236 is expressed with a 6His tag at the N-terminus, 6His tag at the C-terminus.
Names	Guanidinoacetate N-methyltransferase, GAMT, PIG2,TP53I2
Accession #	Q14353
Formulation	Supplied as a 0.2 μm filtered solution of 20mM TrisHCl,1mMDTT,pH 8.0 .
Shipping	The product is shipped on dry ice/ice packs.
Storage	Store at < -20°C, stable for 6 months after receipt. Please minimize freeze-thaw cycles.
Purity	Greater than 95% as determined by reducing SDS-PAGE.
Endotoxin	Less than 0.1 ng/µg (1 IEU/µg) as determined by LAL test.
Amino Acid Sequence	MGSSHHHHHHSSGLVPRGSHMSAPSATPIFAPGENCSPAWGAAPAAYDAADTHLRILGKPVMERW ETPYMHALAAAASSKGGRVLEVGFGMAIAASKVQEAPIDEHWIIECNDGVFQRLRDWAPRQTHKV IPLKGLWEDVAPTLPDGHFDGILYDTYPLSEETWHTHQFNFIKNHAFRLLKPGGVLTYCNLTSWG ELMKSKYSDITIMFEETQVPALLEAGFRRENIRTEVMALVPPADCRYYAFPQMITPLVTKGLEHH HHHH
Background	GAMT is a methyltransferase which belongs to the class I-like SAM-binding methyltransferase superfamily. It contains one RMT2 (arginine N-methyltransferase 2-like) domain and is expressed in liver. GAMT converts guanidoacetate to creatine, using S-adenosylmethionine as the methyl donor. Defects in GAMT are the cause of guanidinoacetate methyltransferase deficiency, which is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, intractable seizures and movement disturbances, severe depletion of creatine/phosphocreatine in the brain, and accumulation of guanidinoacetic acid in brain and body fluids.