2.1.1.2: guanidinoacetate N-methyltransferase
This is an abbreviated version!
For detailed information about guanidinoacetate N-methyltransferase, go to the full flat file.
Reaction
Synonyms
GA methylpherase, GAA methyltransferase, GAMT, guanidinoacetate methyltransferase, guanidinoacetate N-methyltransferase, guanidinoacetate transmethylase, guanidoacetate methyltransferase, methionine-guanidinoacetic transmethylase, MIM 601240, N-guanidinoacetate methyltransferase, S-adenosyl-L-methionine:N-guanidinoacetate methyltransferase
ECTree
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Specific Activity
Specific Activity on EC 2.1.1.2 - guanidinoacetate N-methyltransferase
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0.0006
additional information
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nonradioactive method for measuring enzyme activity in lymphoblasts by using HPLC with UV detector
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L197P transition in exon 6 reduces activity to 0.03 nMol creatine/mg/h, activity is assessed in lymphoblasts
additional information
stable transfection of primary guanidinoacetate N-methyltransferase-deficient fibroblasts with pEGFP-guanidinoacetate N-methyltransferase results in the restoration of guanidinoacetate N-methyltransferase activity to 501 pmol creatine/mg/h from original 1 pmol creatine/mg/h. A 56fold increased guanidinoacetate N-methyltransferase activity is detected in transfected HeLa cells (7700 pmol creatine/mg/h vs. original 137 pmol creatine/mg/h).
additional information
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stable transfection of primary guanidinoacetate N-methyltransferase-deficient fibroblasts with pEGFP-guanidinoacetate N-methyltransferase results in the restoration of guanidinoacetate N-methyltransferase activity to 501 pmol creatine/mg/h from original 1 pmol creatine/mg/h. A 56fold increased guanidinoacetate N-methyltransferase activity is detected in transfected HeLa cells (7700 pmol creatine/mg/h vs. original 137 pmol creatine/mg/h).