產品屬性：
產品名稱 | N-Propionylglycine |
規格 | 25 mg、50 mg、100 mg、250 mg |
貨號 | EY-01Y13499 |
Cas No.: 21709-90-0
別名: N/A
化學名: N/A
分子式: C5H9NO3
分子量: 131.1
溶解度: DMF: 30 mg/ml,DMSO: 30 mg/ml,Ethanol: 15 mg/ml,PBS (pH 7.2): 10 mg/ml
儲存條件: -20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping ConditionEvaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
產品描述：
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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