A branched-chain amino acid
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Isomer(s)
41562Isoleucine34342L-Leucine
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L-Alloisoleucine

Item No. 34904

Product Insert (PDF)Safety Data Sheet (SDS) (PDF)
Technical Information
CAS Number
1509-34-8
Synonyms
  • NSC 206282
Molecular Formula
C6H13NO2
Formula Weight
131.2
Purity
≥95%
Formulation (Request formulation change)
A solid
Solubility(Learn about Variance in Solubility)
DMF: insolDMSO: insolEthanol: insolPBS (pH 7.2): 1 mg/ml
SMILES
O=C([C@H]([C@@H](CC)C)N)O
InChi Code
InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1
InChi Key
AGPKZVBTJJNPAG-UHNVWZDZSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Wet ice in continental US; may vary elsewhere
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    25 mg

    $66

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    50 mg

    $124

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    100 mg

    $182

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    Product Description

    L-Alloisoleucine is a branched-chain amino acid and diastereomer of L-isoleucine.1 It is formed from L-isoleucine by transamination.2 Plasma L-alloisoleucine levels are increased in patients with maple syrup urine disease (MSUD), an inborn error of metabolism characterized by a deficiency in the branched-chain α-ketoacid dehydrogenase complex, the complex that catalyzes the degradation of branched-chain amino acids, leading to the accumulation of branched-chain amino acids in the plasma and urine, a maple syrup-like odor in the urine, and neurological impairments.1

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Schadewaldt, P., Bodner-Leidecker, A., Hammen, H.W., et al. Significance of ʟ-alloisoleucine in plasma for diagnosis of maple syrup urine disease. Clin. Chem. 45(10), 1734-1740 (1999).

    2. Schadewaldt, P., Bodner-Leidecker, A., Hammen, H.W., et al. Formation of L-alloisoleucine in vivo: An L-[13C]isoleucine study in man. Pediatr. Res. 47(2), 271-277 (2000).