Overproduction of N-Acetylneuraminic Acid (Sialic Acid) by Sialuria Fibroblasts

@article{Thomas1985OverproductionON,
  title={Overproduction of N-Acetylneuraminic Acid (Sialic Acid) by Sialuria Fibroblasts},
  author={G. Thomas and L. Reynolds and CAROL S. Miller},
  journal={Pediatric Research},
  year={1985},
  volume={19},
  pages={451-455}
}
ABSTRAC: Fibroblasts from the original sialuria patientare shown to contain high levels of free sialic acid, i.e., anaverage of 87 versus a normal average of 2 nmol/mg ofprotein. Gas liquid chromatography-mass spectrometryanalysis confirmed that the accumulated material is N-acetylneuraminic acid. Addition of D(+) glucosamine (0-5mM) to the media of these cells increased the intracellularfree sialic acid concentrations from 74 to 137 nmol/mgprotein. In contrast, normal cells treated in an… Expand
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Exploration of the Sialic Acid World
Clinical course and biochemistry of sialuria
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