N-Propionylglycine

N-Propionylglycine

CAT N°: 34377
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From 123.00 104.55

N-Propionylglycine is a glycine-conjugated form of propionic acid.{58620} 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.{58620,58621} 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.{58620}

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