规格: | 98% |
分子量: | 131.1 |
包装 | 价格(元) |
25mg | 电议 |
50mg | 电议 |
100mg | 电议 |
250mg | 电议 |
Background:
N-Propionylglycine is a glycine-conjugated form of propionic acid.1It 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.1,2Urinary 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)