A urinary metabolite of leucine
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Isomer(s)
363792-Methylbutyrylglycine
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Isovaleroylglycine

Item No. 17581

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
Formal Name
N-(3-methyl-1-oxobutyl)-glycine
CAS Number
16284-60-9
Synonyms
  • N-Isovalerylglycine
Molecular Formula
C7H13NO3
Formula Weight
159.2
Purity
≥95%
Formulation (Request formulation change)
A crystalline solid
Solubility(Learn about Variance in Solubility)
DMSO: 5 mg/mlMethanol: 1 mg/ml
SMILES
OC(CNC(CC(C)C)=O)=O
InChi Code
InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)
InChi Key
ZRQXMKMBBMNNQC-UHFFFAOYSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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Certificates of Analysis & Batch Specific Data

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    500 mg

    $49

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    1 g

    $82

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    5 g

    $306

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    10 g

    $423

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    Product Description

    Isovaleroylglycine is a compound found in the urine of patients with isovaleric acidemia, a disorder created by defective leucine metabolism due to a deficiency in the isovaleryl coenzyme A dehydrogenase.1 It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid β-oxidation defects.

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Tanaka, K., and Isselbacher, K.J. The isolation and identification of N-isovalerylglycine from urine of patients with isovaleric acidemia. The Journal of Biological Chemisty 242(12), 2966-2972 (1967).