Product description
3-Methylglutaric acid is a metabolite of the essential amino acid L-leucine (Item No. 34342).{58549} It is produced by hydrolysis of the L-leucine catabolic intermediate 3-methylglutaconyl-CoA (3-MG-CoA), which accumulates when the activity of 3-MG-CoA hydratase, the enzyme that converts 3-MG-CoA to HMG-CoA, is deficient, in the mitochondria. 3-Methylglutaric acid (5 mM) inhibits N+/K+-ATPase activity and induces the production of reactive oxygen species (ROS) in rat cortical synaptosomes, as well as induces lipid peroxidation in rat cortical supernatants.{58550,58551} Urinary levels of 3-methylglutaric acid are increased in patients with 3-methylglutaconic aciduria type I, an inborn error of metabolism characterized by mutations in the gene encoding 3-MG-CoA hydratase that can lead to neurological impairments.{58551}
Specifications
CAS number
626-51-7
Supplier
Cayman Chemical
Shipping & storage
Shipping condition
Dry Ice
Storage temperature
-20°C

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