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Biosynthesis of GAA in isolated renal tubules

Guanidinoacetic acid, a precursor of creatine, is an essential substrate for muscle energy metabolism. Since guanidinoacetic acid has been reported to be synthesized from arginine and glycine by glycine amidinotransferase (transamidinase) in kidney homogenates or slices, the purpose of this study was to provide evidence of guanidinoacetic acid synthesis in isolated tubules from rat kidneys, […]

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Formation of arginine and GAA in the kidney in vivo

A method was developed for evaluating the relative rate of conversion of [14C]-citrulline to [14C]arginine in vivo. By this method it was demonstrated that the conversion was almost completely abolished by functional nephrectomy, but not by functional hepatectomy. It was also demonstrated that functional nephrectomy caused a prompt increase in the citrulline concentration in the […]

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Concentration of guanidines in normal human plasma

The concentration of methylguanidine and guanidinosuccinic acid has been measured in normal human plasma and discrepancies between various published methods for measuring methylguanidine have been resolved. Previous methods have been modified to allow these guanidines, together with guanidinoacetic acid and creatine, to be measured on the same serum sample. Mean values in six normal subjects […]

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Formation of creatine from GAA in pancreas

Several investigators established that 2 enzymes are involved in biosynthesis of creatine. Arginine + glycine = guanidinoacetate + ornithine. Guanidinoacetate + S-adenosylmethionine = creatine + S-adenosyl- homocysteine. The first enzyme, arginine- glycine transamidinase, has long been known to occur in mammalian kidney , while the second enzyme, guanidinoacetate methylferase, has been found in liver. [TRUNCATED] […]

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The synthesis of arginine from GAA

Guanidoacetic acid is an important precursor of creatine in the organism. Other than this, little is known regarding the metabolism of this compound. Guanidoacetic acid is formed in the body by the transfer of the amidine group of arginine to glycine according to the following reaction, arginine + glycine = guanidoacetic acid + ornithine, which […]

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The occurrence of GAA in mammalian liver

The synthesis of creatine from guanidinoacetic acid in liver was originally demonstrated by Borsook & Dubnoff, and these authors later concluded that guanidinoacetic acid was not a normal constituent of that organ. This conclusion was confirmed by Bodansky, Duff & McKinney, who reported that no guanidinoacetic acid could be detected in normal animals. [TRUNCATED]. Rosenberg […]

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Urinary GAA, creatine and creatinine in children

The purpose of this report is to record the quantitative urinary excretion of glycocyamine, creatine and total creatinine by a group of normal infants and children. Flood RG, Pinelli RW. Urinary glycocyamine, creatine and creatinine; their excretion by normal infants and children. Am J Dis Child. 1949 Jun;77(6):740-5.

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Synthesis, storage, and excretion of CR, CRN and GAA

The diminished excretion of creatinine in progressive muscular dystrophy is a more striking and specific phenomenon than the excess excretion of creatine, marked though this is. While creatinuria is invariably encountered in all cases of long-standing dystrophy, the extent to which the excretion of creatinine is decreased provides a more reliable indication of the severity […]

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