Fractionation and characterization of urinary heparan sulfate excreted by patients with Sanfilippo syndrome.

@article{Kimura1984FractionationAC,
  title={Fractionation and characterization of urinary heparan sulfate excreted by patients with Sanfilippo syndrome.},
  author={Atsushi P Kimura and Shun-suke Hayashi and Makoto Koseki and Hideo Kochi and K Tsurumi},
  journal={The Tohoku journal of experimental medicine},
  year={1984},
  volume={144 3},
  pages={
          227-36
        }
}
Urinary heparan sulfates (HS) from two siblings with mucopolysaccharidosis (MPS) III-B were fractionated by chromatography with Dowex 1 and Sephadex G-50. Their Mr ranged from 1600 to 8000, and 95% of them were included in the region less than 5000. Fractions with lower Mr contained larger amounts of O-and N-sulfates. The chemical analysis and deaminative cleavage of HS suggested that an intact HS molecule was composed of some blocks rich in GlcNAc and GlcUA and other blocks rich in GlcNS, IdUA… CONTINUE READING

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