Formulation: A solid
Formal Name: 3-methyl-hexanedioic acid
Purity: ≥95%
Formula Markup: C7H12O4
Formula Weight: 160,2
Shelf life (days): 1460
CAS Number: 3058-01-3
Notes: 3-Methyladipic acid is a catabolite of the branched-chain fatty acid phytanic acid (Item No. 90360), which is ingested via the diet.{64606} It is formed from phytanic acid via ω- and then β-oxidation in patients with adult Refsum disease, a disorder characterized by high levels of phytanic acid in the blood and tissues due to a deficiency in phytanoyl-CoA hydroxylase, the enzyme that converts phytanoyl-CoA (Item No. 10011499) to 2-hydroxyphytanoyl-CoA during peroxisomal β-oxidation.{64606,28841,64607} Urinary levels of 3-methyladipic acid have been used as a biomarker for the ω-oxidation of phytanic acid in patients with adult Refsum disease.{64606}