A catabolite of phytanic acid
Related Products
Parent Compound(s)
90360Phytanic Acid
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3-Methyladipic Acid

Item No. 36452

Technical Information
Formal Name
3-methyl-hexanedioic acid
CAS Number
3058-01-3
Synonyms
  • FA 7:1;O2
  • NSC 22069
Molecular Formula
C7H12O4
Formula Weight
Purity
≥95%
A solid
DMF: 14 mg/mlDMSO: 12 mg/mlEthanol: 5 mg/mlPBS (pH 7.2): 5 mg/ml
SMILES
O=C(CCC(CC(O)=O)C)O
InChi Code
InChI=1S/C7H12O4/c1-5(4-7(10)11)2-3-6(8)9/h5H,2-4H2,1H3,(H,8,9)(H,10,11)
InChi Key
SYEOWUNSTUDKGM-UHFFFAOYSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    Product Description

    3-Methyladipic acid is a catabolite of the branched-chain fatty acid phytanic acid (Item No. 90360), which is ingested via the diet.1 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.1,2,3 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.1

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Wierzbicki, A.S., Mayne, P.D., Lloyd, M.D., et alMetabolism of phytanic acid and 3-methyl-adipic acid excretion in patients with adult Refsum disease. J. Lipid Res. 44(8), 1481-1488 (2003).

    2. Verhoeven, N.M., Wanders, R.J., Poll-The, B.T., et alThe metabolism of phytanic acid and pristanic acid in man: A review. J. Inherit. Metab. Dis. 21(7), 697-728 (1998).

    3. Van Veldhoven, P.P. Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism. J. Lipid Res. 51(10), 2863-2895 (2010).