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Category: Amino Acids and Derivatives, Diagnostic, Pharmaceuticals, Intermediates and Fine Chemicals /// Appearance: White Solid /// Application Notes: N-Isobutyrylglycine detection is a useful diagnostic tool in the diagnosis of many organic acidemias and mitochondrial fatty acid beta-oxidation defects. N-Isobutyrylglycine detection is also used in the diagnosis of isobutyryl-CoA dehydrogenase deficiency. /// References: Costa, C. G. et al.: J. Pharmac. Biomed. Anal., 21, 1215 (2000)| Bonafe, L. et al.: Mol. Gen. Metab., 69, 311 (2000)| Sass, J. et al.: J. Inher. Metab. Dis., 27, 741 (2004)|