3-Methylcrotonylglycine-d₂

3-Methylcrotonylglycine-d₂ is the deuterated-labeled 3-Methylcrotonylglycine (HY-113232). 3-Methylcrotonylglycine is an organic acid conjugate associated with leucine catabolism that inhibits respiratory chain complex II-III, mitochondrial creatine kinase, and synaptic membrane Na⁺, K⁺-ATPase. 3-Methylcrotonylglycine increases reactive oxygen species levels to mediate oxidative damage, while inhibiting mitochondrial electron transport, reducing tricarboxylic acid cycle flux, and interfering with intracellular energy transport and neuronal ion homeostasis. 3-Methylcrotonylglycine is a neurotoxic metabolite that accumulates in the metabolic pathway of 3-methylcrotonyl-CoA carboxylase deficiency. 3-Methylcrotonylglycine can be used in studies related to 3-methylcrotonyl-CoA carboxylase deficiency.

• Data Sheet (274 KB)