04513
N-Isobutyrylglycine
analytical standard
Synonym(s):
N-(2-Methyl-1-oxopropyl)glycine, Isobutyryl glycine
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About This Item
Empirical Formula (Hill Notation):
C6H11NO3
CAS Number:
Molecular Weight:
145.16
Beilstein/REAXYS Number:
1762789
MDL number:
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.24
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grade
analytical standard
Quality Level
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
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
Storage Class
11 - Combustible Solids
wgk_germany
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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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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