等级
analytical standard
质量水平
检测方案
≥98.0% (HPLC)
保质期
limited shelf life, expiry date on the label
应用
clinical testing
格式
neat
储存温度
2-8°C
InChI
1S/C8H15NO3/c1-2-3-4-5-7(10)9-6-8(11)12/h2-6H2,1H3,(H,9,10)(H,11,12)
InChI key
UPCKIPHSXMXJOX-UHFFFAOYSA-N
生化/生理作用
Hexanoylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction:acyl-CoA + glycine ↔CoA + N-acylglycine. Hexanoylglycine is a fatty acid metabolite, it appears in the urine of patients with hereditary medium-chain acyl-CoA dehydrogenase (MCAD) deficiency.
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警示用语：
Warning
危险声明
危险分类
Eye Irrit. 2 - Skin Irrit. 2
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
P Rinaldo et al.
Biomedical & environmental mass spectrometry, 18(7), 471-477 (1989-07-01)
We describe a gas chromatographic/mass spectrometric method for the accurate determination of n-hexanoylglycine, 3-phenylpropionylglycine and suberylglycine in urine for the diagnosis of hereditary medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. These acylglycines had previously been detected in urine from patients with MCAD
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