Guanidinoacetate

Guanidinoacetate (GAA) is a metabolite in the urea cycle and the creatine synthesis pathway. It is synthesized from glycine and arginine by the enzyme arginine:glycine amidinotransferase (AGAT). Guanidinoacetate is then converted to creatine by the enzyme guanidinoacetate N-methyltransferase (GAMT).

Biochemical role

Guanidinoacetate serves as a precursor for the production of creatine, an important molecule involved in energy metabolism in muscle and nerve cells. The conversion of guanidinoacetate to creatine is catalyzed by the enzyme guanidinoacetate N-methyltransferase, which transfers a methyl group from S-adenosyl methionine (SAM) to guanidinoacetate.

Clinical significance

Deficiencies in the enzymes AGAT and GAMT can lead to reduced levels of creatine in the brain, resulting in intellectual disability, seizures, and movement disorders. These conditions are known as creatine deficiency syndromes.

See also

• Creatine
• Arginine:glycine amidinotransferase
• Guanidinoacetate N-methyltransferase
• Creatine deficiency syndromes

References

Guanidinoacetate Resources
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