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

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GAMT geneIn BloodProcess of secretionantagonists & inhibitors
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Guanidinoacetate methyltransferase deficiency

Papers overview

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2009
2009
Expanded clinical and molecular spectrum of guanidinoacetate methyltransferase (GAMT) deficiency.
Guanidinoacetate methyltransferase (GAMT) deficiency is a disorder of creatine biosynthesis, characterized by excessive amounts… (More)
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2006
2006
Presymptomatic treatment of neonatal guanidinoacetate methyltransferase deficiency.
Prospective observation in a neonate with guanidinoacetate methyltransferase deficiency (GAMT-D), a severe neurometabolic… (More)
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Highly Cited
2004
Highly Cited
2004
Severely altered guanidino compound levels, disturbed body weight homeostasis and impaired fertility in a mouse model of guanidinoacetate N-methyltransferase (GAMT) deficiency.
We generated a knockout mouse model for guanidinoacetate N-methyltransferase (GAMT) deficiency (MIM 601240), the first discovered… (More)
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Review
2002
Review
2002
Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?
Major achievements made over the last several years have highlighted the important roles of creatine and the creatine kinase… (More)
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2002
2002
Crystal structure of guanidinoacetate methyltransferase from rat liver: a model structure of protein arginine methyltransferase.
Guanidinoacetate methyltransferase (GAMT) is the enzyme that catalyzes the last step of creatine biosynthesis. The enzyme is… (More)
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Highly Cited
2001
Highly Cited
2001
Glycine N -methyltransferase deficiency: A novel inborn error causing persistent isolated hypermethioninaemia
This paper reports clinical and metabolic studies of two Italian siblings with a novel form of persistent isolated… (More)
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Highly Cited
1997
Highly Cited
1997
Creatine deficiency syndrome caused by guanidinoacetate methyltransferase deficiency: diagnostic tools for a new inborn error of metabolism.
Hepatic guanidinoacetate methyltransferase deficiency induces a deficiency of creatine/phosphocreatine in muscle and brain and an… (More)
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1997
1997
Guanidino compounds in guanidinoacetate methyltransferase deficiency, a new inborn error of creatine synthesis.
The first inborn error of creatine metabolism (guanidinoacetate methyltransferase [GAMT] deficiency) has recently been recognized… (More)
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Highly Cited
1996
Highly Cited
1996
Guanidinoacetate methyltransferase deficiency: the first inborn error of creatine metabolism in man.
In two children with an accumulation of guanidinoacetate in brain and a deficiency of creatine in blood, a severe deficiency of… (More)
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Highly Cited
1996
Highly Cited
1996
Creatine replacement therapy in guanidineoacetate methyltransferase deficiency, a novel inborn error of metabolism
BACKGROUND The creatine/creatine-phosphate system is essential for the storage and transmission of phosphate-bound energy in… (More)
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