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 C. Miller},
  journal={Pediatric Research},
  year={1985},
  volume={19},
  pages={451-455}
}
  • G. Thomas, L. Reynolds, C. Miller
  • Published 1985
  • Chemistry, Medicine
  • Pediatric Research
    • 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… CONTINUE READING
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    Abnormal glycosylation with hypersialylated O-glycans in patients with Sialuria.
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    Exploration of the Sialic Acid World
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    N-acetylneuraminic acid accumulation in a buoyant lysosomal fraction of cultured fibroblasts from patients with infantile generalized N-acetylneuraminic acid storage disease.
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    Infantile Sialic Acid Storage Disease: The Fate of Biosynthetically Labeled N-Acetyl-(3H)-Neuraminic Acid in Cultured Human Fibroblasts
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    Role of Nucleotide Sugar Pools in the Inhibition of NCAM Polysialylation by Ammonia
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    Evidence for non‐lysosomal storage of N‐acetylneuraminic acid (sialic acid) in sialuria fibroblasts
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    Clinical course and biochemistry of sialuria
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    The effect ofd-(+)-glucosamine on levels of freeN-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts
    • 1

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