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Glycine propionyl-L-carnitine (GPLC) is a propionyl ester of carnitine that includes an additional glycine component. Due to tissues esterases enzymes, GPLC should act as a prodrug and lead to glycine, carnitine and propionic acid in the body.
L-Carnitine, acetyl-l-carnitine, and propionyl-l-carnitine are available in dietary supplement pills or powders, with a daily amount of 0.5 to 1 g considered to be safe. [1] [3] It is also a drug approved by the Food and Drug Administration to treat primary and certain secondary carnitine-deficiency syndromes secondary to inherited diseases. [1 ...
The second step of L-carnitine biosynthesis requires the 3-hydroxy-N ε-trimethyllysine aldolase enzyme. 3-hydroxy-N ε-trimethyllysine aldolase is a pyridoxal phosphate dependent aldolase, and it catalyses the cleavage of 3-hydroxy-N ε-trimethyllysine into 4-N-trimethylaminobutyraldehyde and glycine.
Carnitine deficiency is found in about 50% of cases. [18] Over 90% of those diagnosed with 3-Methylcrotonyl-CoA carboxylase deficiency by newborn screening remain asymptomatic. The medical abnormalities that present in the few who do show symptoms are not always clearly related to 3-Methylcrotonyl-CoA carboxylase deficiency. [5]
Propionic acidemia is caused by a defect in enzyme called propionyl-CoA carboxylase. Propionic acidemia has an autosomal recessive pattern of inheritance. In healthy individuals, enzyme propionyl-CoA carboxylase converts propionyl-CoA to methylmalonyl-CoA. This is one of many steps in the process of converting certain amino acids and fats into ...
The same set of enzymes is sometimes referred to as glycine synthase when it runs in the reverse direction to form glycine. [2] The glycine cleavage system is composed of four proteins: the T-protein, P-protein, L-protein, and H-protein. They do not form a stable complex, [3] so it is more appropriate to call it a "system" instead of a "complex".
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Glycine propionyl-L-carnitine; H. Harzer; Health issues in athletics; Health issues in youth sports; I. International Journal of Sport Nutrition and Exercise Metabolism;
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