Glutaryl Coenzyme A, Lithium salt
CAS No.: 103192-48-9
Synonym(s): Glutaryl Coenzyme A, Glutaryl-CoA Li salt, Glutaryl-CoA lithium salt
Glutaryl-CoA is a mitochondrial thioester intermediate in the catabolism of lysine, hydroxylysine, and tryptophan. It is primarily converted to crotonyl-CoA and CO₂ by glutaryl-CoA dehydrogenase (GCDH), while an alternative pathway involves peroxisomal oxidation to glutaconyl-CoA and H₂O₂.¹ ² ³ Deficiency of GCDH leads to glutaric aciduria type I (GA-I), characterized by accumulation of glutaryl-CoA and related metabolites.² ³ This accumulation inhibits the α-ketoglutarate dehydrogenase complex (KGDHc), impairing mitochondrial bioenergetics and contributing to striatal damage and encephalopathic crises.²
Applications
Glutaryl-CoA is used in biochemical research to study enzyme mechanisms and substrate specificity of glutaryl-CoA dehydrogenase. Mechanism-based inhibitors such as 2-octynoyl-CoA are employed to probe catalytic residues (e.g., Glu370) and enzyme inactivation.¹ Glutaryl-CoA also serves as a metabolic marker in studies of glutaric aciduria type I and mitochondrial dysfunction.² ³
Other available forms
