04513

N-Isobutyrylglycin

analytical standard

• Synonym(e): N-(2-Methyl-1-oxopropyl)-glycin, Isobutyrylglycin
• Empirische Formel (Hill-System): C₆H₁₁NO₃
• CAS-Nummer: 15926-18-8
• Molekulargewicht: 145.16
• Beilstein: 1762789
• MDL-Nummer: MFCD09043666
• UNSPSC-Code: 12352200
• PubChem Substanz-ID: 329747938
• NACRES: NA.24

Eigenschaften

• Qualität: analytical standard
• Qualitätsniveau: 100
• Assay: ≥95.0% (HPLC)
• Haltbarkeit: limited shelf life, expiry date on the label
• Anwendung(en): clinical testing
• Format: neat
• Lagertemp.: 2-8°C

Beschreibung

Biochem./physiol. Wirkung

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.

Sicherheitshinweise

• Piktogramme: GHS07
• Signalwort: Warning
• H-Sätze: H315,H319
• P-Sätze: P264 - P280 - P302 + P352 - P305 + P351 + P338 - P332 + P313 - P337 + P313
• Gefahreneinstufungen: Eye Irrit. 2 - Skin Irrit. 2
• Lagerklassenschlüssel: 11 - Combustible Solids
• WGK: WGK 3
• Flammpunkt (°F): Not applicable
• Flammpunkt (°C): Not applicable