2-Methylbutyrylglycine is a metabolite of the essential amino acid L-isoleucine. 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.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.
2-Methylbutyrylglycine is a metabolite of the essential amino acid L-isoleucine. 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.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.
分子式
C7H13NO3
分子量
159.2
CAS号
52320-67-9
中文名称
N-(2-甲基-1-氧代丁基)-甘氨酸
运输条件
Room temperature in continental US; may vary elsewhere.