17/02/2018 Creatine metabolism and the urea cycle.

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Mol Genet Metab. 2010;100 Suppl 1:S49-52. doi: 10.1016/j.ymgme.2010.02.020. Epub 2010 Mar 1.

Creatine metabolism and the urea cycle.

Brosnan JT1, Brosnan ME.

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Abstract
Because creatine and creatine phosphate are irreversibly converted to creatinine, there is a
continuous need for their replacement. This occurs by means of diet and de novo synthesis.
Dietary creatine is provided in animal products and can amount to about half of the required
amount. Synthesis provides the remainder. Creatine synthesis is a major component of arginine
metabolism, amounting to more than 20% of the dietary intake of this amino acid. Creatine
metabolism is of importance to patients with urea cycle disorders in two ways, both related to
arginine levels. In patients with arginase deficiency, markedly elevated arginine levels may result
in higher concentrations of guanidinoacetate and higher rates of creatine synthesis. This is of
concern because it is thought that elevated levels of guanidinoacetate may exert neurotoxic
effects. In the case of the other urea cycle disorders, arginine levels are markedly decreased
unless the patients are supplemented with this amino acid. Decreased levels of arginine may
result in decreased rates of creatine synthesis. This may be compounded by the fact that such
patients, maintained on low protein diets, will also have lower dietary creatine intakes. There is
some evidence that this may decrease brain creatine levels which may contribute to the
neurological symptoms exhibited by these patients. It is clear that patients with urea cycle
disorders also have altered creatine metabolism. Whether this contributes in a significant way to
their neurological symptoms remains an open question.

PMID: 20304692 DOI: 10.1016/j.ymgme.2010.02.020

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https://www.ncbi.nlm.nih.gov/pubmed/20304692 1/2
17/02/2018 Creatine metabolism and the urea cycle. - PubMed - NCBI

https://www.ncbi.nlm.nih.gov/pubmed/20304692 2/2