Corpus ID: 12348750

On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease.

@article{Schadewaldt1990OnTM,
  title={On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease.},
  author={Peter Schadewaldt and H. -W. Hammen and C. Dalle-Feste and Udo Wendel},
  journal={Journal of inherited metabolic disease},
  year={1990},
  volume={13 2},
  pages={
          137-50
        }
}
L-Alloisoleucine formation from L-isoleucine was studied in vitro and in vivo. When a healthy subject was loaded with L-isoleucine, plasma levels of L-isoleucine and 3-methyl-2-oxopentanoate (KMV), as well as L-alloisoleucine, increased. Peak values were reached successively and were in the order L-isoleucine much greater than KMV much greater than L-alloisoleucine. Metabolic clearance of L-isoleucine and KMV was rapid; clearance of L-alloisoleucine was considerably delayed. When human skin… Expand
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