3-Methyl<wbr/>adipic Acid

3-Methyladipic Acid

CAT N°: 36452
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From 43.00 36.55

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 ?- and then ?-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 ?-oxidation.{64606,28841,64607} Urinary levels of 3-methyladipic acid have been used as a biomarker for the ?-oxidation of phytanic acid in patients with adult Refsum disease.{64606}

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