Formulation: A solid
Formal Name: N-(2-methyl-1-oxobutyl)-glycine
Purity: ≥95%
Formula Markup: C7H13NO3
Formula Weight: 159,2
Shelf life (days): 1460
CAS Number: 52320-67-9
Notes: 2-Methylbutyrylglycine is a metabolite of the essential amino acid L-isoleucine.{42603,58621} It is produced by hydrolysis of the L-isoleucine catabolic intermediate (S)-2-methylbutyryl-CoA, which accumulates when the activity of 2-methylbutyryl-CoA dehydrogenase is deficient. 2-Methylbutyrylglycine (0.5-5 mM) induces the formation of thiobarbituric acid reactive substances (TBARS) in isolated rat cerebral cortex.{65151} Elevated levels of 2-methylbutyrylglycine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency.{42603,58621}