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2. Glycine propionyl-L-carnitine - Wikipedia

   en.wikipedia.org/wiki/Glycine_propionyl-l-carnitine

   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.

3. Propionic acidemia - Wikipedia

   en.wikipedia.org/wiki/Propionic_acidemia

   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 ...

4. Carnitine - Wikipedia

   en.wikipedia.org/wiki/Carnitine

   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 ...

5. 3-Methylcrotonyl-CoA carboxylase deficiency - Wikipedia

   en.wikipedia.org/wiki/3-Methylcrotonyl-CoA...

   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]

6. Carnitine biosynthesis - Wikipedia

   en.wikipedia.org/wiki/Carnitine_biosynthesis

   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.

7. Glutaric acidemia type 2 - Wikipedia

   en.wikipedia.org/wiki/Glutaric_acidemia_type_2

   [6] [7] Depending on the subtype, riboflavin [7] (100-400 mg/day), [6] coenzyme Q10 (CoQ10), [6] L-carnitine, [7] or glycine [7] supplements may be used to help restore energy production. Some small, uncontrolled studies [ 8 ] [ 9 ] [ 10 ] have reported that racemic salts of beta-hydroxybutyrate (one of the ketone bodies ) were helpful in ...