Citrulline supplementation

The cause is defective transport of dibasic amino acids by the proximal tubule and intestine. The transport defect occurs at the basolateral rather than the luminal membrane. Hyperammonemia reflects a deficiency of intra-mitochondrial ornithine. An effective treatment is oral citrulline supplementation, which corrects the hyperammonemia by allowing replenishment of the mitochondrial pool of ornithine. 

Patients who survive the neonatal period can be maintained with a low-protein diet and sodium benzoate. A useful therapeutic adjunct for citrullinemia and argininosuccinic aciduria is dietary arginine supplementation, which enhances the ability to eliminate nitrogen as either citrulline or argininosuccinate. Maintaining normal arginine levels also facilitates protein synthesis. 

CFS patients and is related to autonomic dysfunction. In a separate rat study, Giannesini et al. used citrulline malate (CM) to treat asthenia and found that the supplementation prevented the basal PCr/ATP ratio reduction and normalized the pHi time-course during muscular activ-ity. They conclude that CM supplementation corrects the impaired control of oxidative function and has protective effect on basal energy metabolism. The data from either human or animal studies provide a potential approach to therapy. 

Enzyme inhibition can be reversed by supplementation of arginine or citrulline.34 106 Pathovars of P. syringae were shown to inhibit Escherichia coli growth, an effect reversed by L-arginine, but not by L-citrulline or L-glutamine.121 This suggested that the site of action of the toxin produced is involved in the conversion of citrulline to arginine in the urea cycle. 

Supplement Essential amino acids, arginine in ASS and ASL deficiency, and citrulline in OTC and CPS deficiency Toxic Ammonia in aU UCD Argininosuccinic acid in ASL deficiency Arginine in arginase deficiency... 

The effects of oral ingestion of citrulline malate on the bioenergetics and mechanical performance of muscle in the normal rat have been investigated with P MRS. Supplementation with citrulline malate did not affect the ratio of phosphocreatine/ATP (PCr/ATP), the concentration of phos-phorylated metabolites or intracellular pH at rest. During the stimulation, it lead to a 23% enhancement of specific force production, but had no effect on the time-courses of phosphorylated metabolites or intracellular pH. 

Citrulline Synthesis. The first syntheses of citrulline and arginine by the addition of NHa and CO2 to ornithine were achieved in the laboratory of Cohen, where soluble enzymes of liver supplemented with cell particles were found to carry out these syntheses. The soluble extract was found to contain the several activities involved in the reactions of the urea cycle, while the particles were found to function solely as a source of ATP. The synthesis of citrulline was found to require ornithine, CO2, NH3, and... 

Hayashi, T, P.A.R. Juliet, H. Matsui-Hirai, A. Miyazaki, A. Fukatsu, J. Funami, A. Iguehi, and L.J. Ignarro, 2005. L-citrulline and 1-arginine supplementation retards the progression of high-eholesterol-diet-indueed atherosclerosis in rabbits. Proc. Natl. Acad. Sci. U.S.A. 102, 13681-13686. 

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