04513
N-Isobutyrylglycine
analytical standard
别名:
N-(2-Methyl-1-oxopropyl)glycine, Isobutyryl glycine
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关于此项目
经验公式（希尔记法）:
C6H11NO3
化学文摘社编号:
分子量:
145.16
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.24
Beilstein/REAXYS Number:
1762789
MDL number:
grade
analytical standard
assay
≥95.0% (HPLC)
shelf life
limited shelf life, expiry date on the label
application(s)
clinical testing
format
neat
storage temp.
2-8°C
Quality Level
Biochem/physiol Actions
Isobutyrylglycine 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. Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine.
signalword
Warning
hcodes
Hazard Classifications
Eye Irrit. 2 - Skin Irrit. 2
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
J O Sass et al.
Journal of inherited metabolic disease, 27(6), 741-745 (2004-10-27)
Isobutyryl-CoA dehydrogenase (IBD) is an enzyme involved in the catabolism of the branched-chain amino acid valine. We report a third and a fourth child with IBD deficiency who were both detected during newborn screening with tandem mass spectrometry and so
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