Product description
a-Ketoisocaproic acid is a metabolite of L-leucine (Item No. 34342) catabolism.{60680} It increases the production of reactive species and decreases the activity of mitochondrial complex I, also known as NADH dehydrogenase, and complex II-III in the rat hippocampus when administered at an intracerebroventricular dose of 4 µmol.{60681} Urine levels of a-ketoisocaproic acid are reduced in db/db diabetic mice compared with heterozygous db/m non-diabetic mice.{60682} a-Ketoisocaproic acid accumulates in the tissues and body fluids of patients with maple syrup urine disease, an inborn error of metabolism characterized by branched-chain a-keto acid dehydrogenase (BCKAD) deficiency and leads to progressive ketoacidosis, failure to thrive, neurological dysfunction, and, potentially, death.{60681}
Specifications
CAS number
816-66-0
Supplier
Cayman Chemical
Shipping & storage
Shipping condition
Dry Ice
Storage temperature
-20°C

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