For research use only. Not for therapeutic Use.
L-allo-Isoleucine(cas 1509-34-8) may be used in studies on maple syrup urine disease (MSUD). In patients with classical MSUD (n = 7), alloisoleucine was beyond the cutoff value in 2451 of 2453 unselected samples. In patients with variant MSUD (n = 9), alloisoleucine was >5 micromol/L in all samples taken for establishment of diagnosis and in 94% of the samples taken for treatment control (n = 624). With the other branched-chain amino acids, the frequency of diagnostically significant increases was <45%. The plasma L-alloisoleucine above the cutoff value of 5 micromol/L is the most specific and most sensitive diagnostic marker for all forms of MSUD.
| Catalog Number | M050436 |
| CAS Number | 1509-34-8 |
| Synonyms | (2S,3R)-2-amino-3-methylpentanoic acid; L-allo-Isoleucine; L-Alloisoleucine; |
| Molecular Formula | C6H13NO2 |
| Purity | ≥95% |
| Target | Endogenous Metabolite |
| Storage | -20°C |
| IUPAC Name | (2S,3R)-2-amino-3-methylpentanoic acid |
| InChI | 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 |
| InChIKey | AGPKZVBTJJNPAG-UHNVWZDZSA-N |
| SMILES | CCC(C)C(C(=O)O)N |
| Reference | <br /> |
