N-Propionylglycine (Synonyms: NSC 158539) |
| Catalog No.GC49240 |
A glycine-conjugated form of propionic acid
Products are for research use only. Not for human use. We do not sell to patients.
Cas No.: 21709-90-0
Sample solution is provided at 25 µL, 10mM.
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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
1.Riemersma, M., Hazebroek, M.R., Helderman-van den Enden, A.T.J.M., et al.Propionic acidemia as a cause of adult-onset dilated cardiomyopathyEur. J. Hum. Genet.25(11)1195-1201(2017) 2.Fong, B.M.-W., Tam, S., and Leung, S.-Y.Quantification of acylglycines in human urine by HPLC electrospray ionization-tandem mass spectrometry and the establishment of pediatric reference interval in local ChineseTalanta88193-200(2012)
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