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

  title={Overproduction of N-Acetylneuraminic Acid (Sialic Acid) by Sialuria Fibroblasts},
  author={G. Thomas and L. Reynolds and C. Miller},
  journal={Pediatric Research},
  • 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
    24 Citations
    Abnormal glycosylation with hypersialylated O-glycans in patients with Sialuria.
    • 22
    Exploration of the Sialic Acid World
    • 43
    Clinical course and biochemistry of sialuria
    • 25


    The thiobarbituric acid assay of sialic acids.
    • L. Warren
    • Chemistry, Medicine
    • The Journal of biological chemistry
    • 1959
    • 5,293
    • PDF
    Human liver glycoprotein sialyltransferase.
    • 21
    Isolation and identification of 2-deoxy-2,3-dehydro-N-acetylneuraminic acid from the urine of a patient with sialuria.
    • 49
    The sialic acids. X. Purification and properties of cytidine 5'-monophosphosialic acid synthetase.
    • 96
    • PDF
    Glucosamine metabolism in regenerating rat liver.
    • 15