Creatine and guanidinoacetate content of human milk and infant formulas: implications for creatine deficiency syndromes and amino acid metabolism

Creatine, central nervous system and creatine deficiency syndromes

It was long thought that most of brain creatine was of peripheral origin. However, recent works have demonstrated that creatine crosses blood-brain barrier only with poor efficiency, and that CNS must ensure parts of its creatine needs by its own creatine synthesis pathway, thank to the brain expression of AGAT and GAMT (creatine synthesis) and SLC6A8 (creatine transporter). This new understand...

Creatine Deficiency Syndromes

Creatine metabolism and disease Primary creatine metabolism disorders- Cerebral Creatine Deficiency Syndromes Cerebral Creatine Deficiency Syndromes (CCDS) are a group of disorders consisting of defects in proteins involved in creatine biosynthesis, AGAT and GAMT, and in its transporter, SLC6A8

Creatine synthesis: hepatic metabolism of guanidinoacetate and creatine in the rat in vitro and in vivo.

Since creatinine excretion reflects a continuous loss of creatine and creatine phosphate, there is a need for creatine replacement, from the diet and/or by de novo synthesis. Creatine synthesis requires three amino acids, methionine, glycine, and arginine, and two enzymes, l-arginine:glycine amidinotransferase (AGAT), which produces guanidinoacetate acid (GAA), and guanidinoacetate methyltransf...

Free amino acid content in infant formulas

Purpose – Infant formula is the sole nutrition and food source for many infants. Information on the free amino acid (FAA) content of formulas, particularly those that are protein hydrolysate based, is limited, despite emerging evidence for the role of FAAs in regulating eating behaviour. The purpose of this paper is to measure levels of essential, semi-essential, and nonessential FAAs in commer...