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
    View on Springer
    nature.com
    24 Citations
    Background Citations
    5
    24 Citations
    Citation Type

    Citation Type

    Accumulation of N-acetylneuraminic acid (sialic acid) in human fibroblasts cultured in the presence of N-acetylmannosamine.
    • 19
    Accumulation of [3H]Sialyl-conjugates in sialidosis (sialidase-deficient) fibroblasts cultured in the presence of [3H]-N-acetylmannosamine
    • 1
    Abnormal glycosylation with hypersialylated O-glycans in patients with Sialuria.
    • 22
    Exploration of the Sialic Acid World
    • 42
    N-acetylneuraminic acid accumulation in a buoyant lysosomal fraction of cultured fibroblasts from patients with infantile generalized N-acetylneuraminic acid storage disease.
    • 15
    Infantile Sialic Acid Storage Disease: The Fate of Biosynthetically Labeled N-Acetyl-(3H)-Neuraminic Acid in Cultured Human Fibroblasts
    • 7
    • PDF
    Role of Nucleotide Sugar Pools in the Inhibition of NCAM Polysialylation by Ammonia
    • 36
    Evidence for non‐lysosomal storage of N‐acetylneuraminic acid (sialic acid) in sialuria fibroblasts
    • 5
    Clinical course and biochemistry of sialuria
    • 25
    The effect ofd-(+)-glucosamine on levels of freeN-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts
    • 1

    References

    SHOWING 1-10 OF 24 REFERENCES
    Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis II) fibroblasts.
    • 90
    The thiobarbituric acid assay of sialic acids.
    • L. Warren
    • Chemistry, Medicine
    • The Journal of biological chemistry
    • 1959
    • 5,289
    • PDF
    Human liver glycoprotein sialyltransferase.
    • 21
    [31] CMP-sialic acid synthetase (cytidine-5′-monophospho-sialic acid synthetase)
    • 41
    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
    Mucolipidosis III (Pseudo-Hurler Polydystrophy): Multiple Lysosomal Enzyme Abnormalities in Serum and Cultured Fibroblast Cells
    • 63
    • PDF
    Alterations in Cultured Fibroblasts of Sibs with an Infantile Form of a Free (Unbound) Sialic Acid Storage Disorder
    • 25
    • PDF
    Uridine diphosphate N-acetyl-D-glucosamine-2-epimerase from rat liver. I. Catalytic and regulatory properties.
    • 34
    Glucosamine metabolism in regenerating rat liver.
    • 15