Formation of L-Alloisoleucine In Vivo : An L-[13C]Isoleucine Study in Man

@article{Schadewaldt2000FormationOL,
  title={Formation of L-Alloisoleucine In Vivo : An L-[13C]Isoleucine Study in Man},
  author={P. Schadewaldt and A. Bodner-Leidecker and H. Hammen and U. Wendel},
  journal={Pediatric Research},
  year={2000},
  volume={47},
  pages={271-271}
}
L-Alloisoleucine (2 S, 3 R), a diastereomer of L-isoleucine (2 S, 3 S), is a normal constituent of human plasma. Considerable amounts accumulate in maple syrup urine disease, in which the branched-chain 2-oxo acid dehydrogenase step is impaired. The mechanism of L-alloisoleucine formation, however, is unclear. We addressed this issue by performing oral L-[1-13C]isoleucine loading (38 μmol/kg body wt, 50% 1-13C) in overnight-fasted healthy subjects (n = 4) and measuring the 3-h kinetics of 13C… Expand
Deciphering the Biosynthetic Origin of L-allo-Isoleucine.
Initial catabolic steps of isoleucine, the R-pathway and the origin of alloisoleucine.
  • O. Mamer
  • Chemistry, Medicine
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Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation
Expanding the genetic and phenotypic spectrum of branched‐chain amino acid transferase 2 deficiency
Analysis of plasma amino acids by HPLC with photodiode array and fluorescence detection.
Newborn screening and inborn errors of metabolism
  • M. Pasquali, N. Longo
  • Medicine
  • American journal of medical genetics. Part C, Seminars in medical genetics
  • 2011

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