L-allo-Isoleucine 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. References: Rodrigues KT, Mekahli D, Tavares MF, Van Schepdael A. Development and validation of a CE-MS method for the targeted assessment of amino acids in urine. Electrophoresis. 2016 Apr;37(7-8):1039-47. doi: 10.1002/elps.201500534. Epub 2016 Feb 25. PubMed PMID: 26826549.

纯度：≥98%

CAS：1509-34-8