04513

Supelco

N-Isobutyrylglycine

analytical standard

Synonym(s):
N-(2-Methyl-1-oxopropyl)glycine, Isobutyryl glycine
Empirical Formula (Hill Notation):
C6H11NO3
CAS Number:
Molecular Weight:
145.16
Beilstein/REAXYS Number:
1762789
MDL number:
PubChem Substance ID:
NACRES:
NA.24
Pricing and availability is not currently available.

grade

analytical standard

Quality Level

assay

≥95.0% (HPLC)

shelf life

limited shelf life, expiry date on the label

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.

Pictograms

Exclamation mark

Signal Word

Warning

Hazard Statements

Precautionary Statements

RIDADR

NONH for all modes of transport

WGK Germany

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificate of Analysis
Certificate of Origin
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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