Sanfilippo Disease Type B: Enzyme Replacement and Metabolic Correction in Cultured Fibroblasts

DOI Web Site 被引用文献2件
  • J. S. O'Brien
    Department of Neurosciences, School of Medicine, University of California, San Diego, La Jolla 92037
  • A. L. Miller
    Department of Neurosciences, School of Medicine, University of California, San Diego, La Jolla 92037
  • A. W. Loverde
    Department of Neurosciences, School of Medicine, University of California, San Diego, La Jolla 92037
  • M. L. Veath
    Department of Neurosciences, School of Medicine, University of California, San Diego, La Jolla 92037

抄録

<jats:p>α-Acetylglucosaminidase, purified from human placenta, corrected the defect in mucopolysaccharide degradation when added to cultured fibroblasts from patients with Sanfilippo disease type B. A small cellular concentration of enzyme gave a large corrective effect. The half-life of disappearance of enzyme activity was 4 to 7 days.</jats:p>

収録刊行物

  • Science

    Science 181 (4101), 753-755, 1973-08-24

    American Association for the Advancement of Science (AAAS)

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