Product Information
- Glycine
- N-[3-(methyl-d3)-1-oxobutyl-2,2,3,4,4,4-d6]-
- N-(3-(Methyl-d3)-1-oxobutyl-2,2,3,4,4,4-d6)glycine
- Isovalerylglycine-d9
- N-Isovaleroylglycine-d9
- N-Isovalerylglycined-d9
- N-(3-Methylbutanoyl)glycine-D9 (3-Methylbutanoyl-D9)
Applications Isotope labelled N-Isovalerylglycine is a useful diagnostic tool in the diagnosis of many organic acidemias and mitochondrial fatty acid β-oxidation defects. N-Isovalerylglycine is found in the urine of patients with isovaleric acidemia, an inborn error of leucine metabolism.
References Tanaka, K. et al.: J. Biol. Chem., 242, 2966 (1967); Bonafe, L. et al.: Mol. Gen. Metab., 69, 311 (2000);
Chemical properties
Technical inquiry about: TR-I917602 N-Iso Valerylglycine-d9
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