A metabolite of isoleucine
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2-Methylbutyrylglycine

Item No. 36379

Technical Information
Formal Name
N-(2-methyl-1-oxobutyl)-glycine
CAS Number
52320-67-9
Synonyms
  • 2-MBG
  • N-sec-Valerylglycine
Molecular Formula
C7H13NO3
Formula Weight
Purity
≥95%
A solid
DMSO: SolubleEthanol: Soluble
SMILES
O=C(CNC(C(CC)C)=O)O
InChi Code
InChI=1S/C7H13NO3/c1-3-5(2)7(11)8-4-6(9)10/h5H,3-4H2,1-2H3,(H,8,11)(H,9,10)
InChi Key
HOACIBQKYRHBOW-UHFFFAOYSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    Product Description

    2-Methylbutyrylglycine is a metabolite of the essential amino acid L-isoleucine.1,2 It is produced by hydrolysis of the L-isoleucine catabolic intermediate (S)-2-methylbutyryl-CoA, which accumulates when the activity of 2-methylbutyryl-CoA dehydrogenase is deficient. 2-Methylbutyrylglycine (0.5-5 mM) induces the formation of thiobarbituric acid reactive substances (TBARS) in isolated rat cerebral cortex.3 Elevated levels of 2-methylbutyrylglycine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency.1,2

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Gibson, K.M., Burlingame, T.G., Hogema, B., et al2-Methylbutyryl-coenzyme A dehydrogenase deficiency: A new inborn error of L-isoleucine metabolism. Pediatr. Res. 47(6), 830-833 (2000).

    2. Fong, B.M.-W., Tam, S., and Leung, S.-Y. Quantification of acylglycines in human urine by HPLC electrospray ionization-tandem mass spectrometry and the establishment of pediatric reference interval in local Chinese. Talanta 88, 193-200 (2012).

    3. Knebel, L.A., Zanatta, Â., Tonin, A.M., et al2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain. Brain Res. 1478, 74-82 (2012).