Cellular bioenergetics of guanidinoacetic acid: the role of mitochondria
- 247 Downloads
Guanidinoacetic acid (GAA) is a natural precursor of creatine, and a possible substrate for the creatine kinase (CK) enzyme system, serving as a creatine mimetic. Its direct role in cellular bioenergetics has been confirmed in several studies, however GAA utilization by CK seems to be a second-rate as compared to creatine, and compartment-dependent. Here we discuss various factors that might affect GAA use in high-energy phosphoryl transfer in the cytosol and mitochondria.
KeywordsCreatine kinase Guanidinoacetic acid Creatine Mitochondria Bioenergetics
This work was supported by the Serbian Ministry of Science (Grant No. 175037).
Conflict of interest
The author declares no conflicts of interest related to this work.
- Lygate CA, Aksentijevic D, Dawson D, ten Hove M, Phillips D, de Bono JP, Medway DJ, Sebag-Montefiore L, Hunyor I, Channon KM, Clarke K, Zervou S, Watkins H, Balaban RS, Neubauer S (2013) Living without creatine: unchanged exercise capacity and response to chronic myocardial infarction in creatine-deficient mice. Circ Res 112:945–955CrossRefGoogle Scholar
- Schmidt A, Marescau B, Boehm EA, Renema WK, Peco R, Das A, Steinfeld R, Chan S, Wallis J, Davidoff M, Ullrich K, Waldschütz R, Heerschap A, De Deyn PP, Neubauer S, Isbrandt D (2004) Severely altered guanidino compound levels, disturbed body weight homeostasis and impaired fertility in a mouse model of guanidinoacetate N-methyltransferase (GAMT) deficiency. Hum Mol Genet 13:905–921CrossRefGoogle Scholar
- Wyss M, Kaddurah-Daouk R (2000) Creatine and creatinine metabolism. Physiol Rev 80:1107–1213Google Scholar