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

@article{Thomas1985OverproductionON,
  title={Overproduction of N-Acetylneuraminic Acid (Sialic Acid) by Sialuria Fibroblasts},
  author={George H. Thomas and Linda W. 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… CONTINUE READING

Citations

Publications citing this paper.
SHOWING 1-10 OF 11 CITATIONS

Free sialic acid storage disease

  • European Journal of Pediatrics
  • 1987

References

Publications referenced by this paper.
SHOWING 1-10 OF 24 REFERENCES

2 - Acetamidoelucal . a new metabolite isolated from the 1 lr ; np nf 9 - . . . A - - . - patient with sialuria

JP Kamerling, G Strecker, +3 authors Vliegenthart JFG
  • 1979

Isolation and identification of 2deoxy-2,3dehydro-N-acetylneuraminicacid from the urine of a patient with sialuria

JP Kamerling, JFG Vliegenthart, R Schauer, G Strecker, J Montreuil
  • Eur J Biochem
  • 1975
VIEW 2 EXCERPTS