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N-Propionylglycine is a glycine-conjugated form of propionic acid.1 It is produced by glycine-N-acylase from propionyl-CoA, a catabolic intermediate that accumulates when the activity of propionyl-CoA carboxylase, the enzyme that converts propionyl-CoA to methylmalonyl-CoA, is deficient, in the mitochondria.1,2 Urinary levels of N-propionylglycine are increased in patients with propionic acidemia, an inborn error of metabolism characterized by vomiting, lethargy, protein intolerance, and failure to thrive.1
WARNING This product is not for human or veterinary use.
1. Propionic acidemia as a cause of adult-
2. Quantification of acylglycines in human urine by HPLC electrospray ionization-