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
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Source Tissue
Source Tissue on EC 2.1.1.2 - guanidinoacetate N-methyltransferase
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weak GAMT expression is detected in the epithelium of alimentary tract
in embryos at 12.5 days of gestation guanidinoacetate N-methyltransferase is detectable in the hepatic primordium only, with all other tissues being negative. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies
in embryos at 18.5 days of gestation guanidinoacetate N-methyltransferase is detectable. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies
GAMT is strongly expressed in all neurons and glia cells of neural cord
GAMT is highly expressed in perivascular pericytes and smooth muscle cells after birth
GAMT was highly expressed in perivascular pericytes and smooth muscle cells after birth
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L197P mutation in exon 6: guanidinoacetate increases to 2566 microMol/l, creatine decreases to 3.7 microMol/l
additional information
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L197P mutation in exon 6: guanidinoacetate increases to 33.6 microMol/l, creatine decreases to 24 microMol/l
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high expression in oligodendrocytes and olfactory ensheathing glia, moderate expression in astrocytes, low expression in neurons and microglia
two waves of high GAMT expression. The first involves high expression in mitotic cells in the ventricular zone of the brain wall and the external granular layer of the cerebellum at the embryonic and neonatal stages. The second is initiated by striking up-regulation of GAMT in oligodendrocytes during the second and third postnatal weeks and continues to adulthood
all parts of brain, neurons and glia, very low levels in astrocytes
in embryos at 15.5 days of gestation guanidinoacetate N-methyltransferase is detectable in pons and striatum. In embryos at 18.5 days of gestation guanidinoacetate N-methyltransferase is detectable in neocortex, hippocampus, striatum, pallidum and spinal cord. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies
in embryos at 15.5 and 18.5 days of gestation guanidinoacetate N-methyltransferase is detectable in liver. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies
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L197P mutation in exon 6, reduces the activity of guanidinoacetate N-methyltransferase
GAMT levels are low to moderate in neuroblasts residing in the ventricular zone, increase during the second postnatal week, and decrease to very low levels thereafter
striking up-regulation of GAMT in oligodendrocytes during the second and third postnatal weeks, expression continues to adulthood
in embryos at 15.5 and 18.5 days of gestation guanidinoacetate N-methyltransferase is detectable in pancreas. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies
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the piglet pancreatic GAMT activity is only 1% of that found in the liver
in embryos at 18.5 days of gestation guanidinoacetate N-methyltransferase is detectable. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies
additional information
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absent in brain, small intestine, seminal vesicle, lung, vas deferens, cauda epididymis, coagulating gland and spleen
additional information
guanidinoacetate N-methyltransferase can not be detected in kidney of embryos of 12.5, 15.5 and 18.5 days of gestation. Non-radioactive in situ hybridization (58°C for 40 h in 5 x SSC, 50% formamide and 40 microg/ml salmon sperm DNA) with digoxigenin-labeled antisense and sense riboprobes (400 ng/ml) for rat guanidinoacetate N-methyltransferase. guanidinoacetate N-methyltransferase proteins are detected with rabbit polyclonal antibodies