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Guanidinoacetate N-Methyltransferase

Known as: Guanidinoacetate N-Methyltransferase [Chemical/Ingredient], GA Methyltransferase, Guanidinoacetate N Methyltransferase 
This enzyme catalyzes the last step of CREATINE biosynthesis by catalyzing the METHYLATION of guanidinoacetate to CREATINE.
National Institutes of Health

Related topics

Related topics

10 relations
GAMT geneIn BloodProcess of secretionantagonists & inhibitors

Narrower (2)

Gamt protein, ratGuanidinoacetate methyltransferase deficiency

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2014
Highly Cited
2014
Guanidinoacetate methyltransferase (GAMT) deficiency: outcomes in 48 individuals and recommendations for diagnosis, treatment and monitoring.
2013
2013
Elevation of guanidinoacetate in newborn dried blood spots and impact of early treatment in GAMT deficiency.
Highly Cited
2006
Highly Cited
2006
Presymptomatic treatment of neonatal guanidinoacetate methyltransferase deficiency
Prospective observation in a neonate with guanidinoacetate methyltransferase deficiency (GAMT-D), a severe neurometabolic… 
Highly Cited
2004
Highly Cited
2004
Distinct cellular expressions of creatine synthetic enzyme GAMT and creatine kinases uCK‐Mi and CK‐B suggest a novel neuron–glial relationship for brain energy homeostasis
The creatine/phosphocreatine shuttle system, as catalysed reversibly by creatine kinases, is thought to be essential for the… 
2004
2004
Phosphorylated guanidinoacetate partly compensates for the lack of phosphocreatine in skeletal muscle of mice lacking guanidinoacetate methyltransferase
The effects of creatine (Cr) absence in skeletal muscle caused by a deletion of guanidinoacetate methyltransferase (GAMT) were… 
Highly Cited
2004
Highly Cited
2004
Creatine and guanidinoacetate: diagnostic markers for inborn errors in creatine biosynthesis and transport.
Highly Cited
2000
Highly Cited
2000
Brain creatine depletion: Guanidinoacetate methyltransferase deficiency (improving with creatine supplementation)
Article abstract The authors describe an Italian child with guanidinoacetate methyltransferase deficiency, neurologic regression… 
Highly Cited
1998
Highly Cited
1998
An accurate stable isotope dilution gas chromatographic-mass spectrometric approach to the diagnosis of guanidinoacetate methyltransferase deficiency.
Highly Cited
1996
Highly Cited
1996
Creatine replacement therapy in guanidineoacetate methyltransferase deficiency, a novel inborn error of metabolism
Highly Cited
1972
Highly Cited
1972
Distribution of creatine, guanidinoacetate and the enzymes for their biosynthesis in the animal kingdom. Implications for phylogeny.
1. The distribution of creatine and the creatine-synthesizing enzymes in the animal kingdom has been investigated. Creatine was… 
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