Synthesis and degradation of hyaluronic acid in the cultured fibroblasts of Marfan's disease.

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  • Author(s): Lamberg SI; Dorfman A
  • Source:
    The Journal of clinical investigation [J Clin Invest] 1973 Oct; Vol. 52 (10), pp. 2428-33.
  • Publication Type:
    Journal Article
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: American Society for Clinical Investigation Country of Publication: United States NLM ID: 7802877 Publication Model: Print Cited Medium: Print ISSN: 0021-9738 (Print) Linking ISSN: 00219738 NLM ISO Abbreviation: J Clin Invest Subsets: MEDLINE
    • Publication Information:
      Publication: 1999- : Ann Arbor, MI : American Society for Clinical Investigation
      Original Publication: New Haven [etc.] American Society for Clinical Investigation.
    • Subject Terms:
      Hyaluronic Acid/*metabolism ; Marfan Syndrome/*metabolism; Acetates/metabolism ; Adult ; Animals ; Carbon Isotopes ; Cattle ; Cetylpyridinium ; Cycloheximide/pharmacology ; Female ; Fibroblasts/drug effects ; Fibroblasts/enzymology ; Fibroblasts/metabolism ; Glucosamine/metabolism ; Glycosaminoglycans/analysis ; Glycosaminoglycans/metabolism ; Glycoside Hydrolases/analysis ; Humans ; Hyaluronic Acid/analysis ; Hyaluronic Acid/biosynthesis ; Kinetics ; Male ; Marfan Syndrome/enzymology ; Molecular Weight ; Mucopolysaccharidoses/metabolism ; Staining and Labeling ; Time Factors
    • Abstract:
      Increased amounts of hyaluronic acid accumulate in fibroblasts cultured from patients with Marfan's disease, an autosomal dominant disorder. In the recessive Hurler's disease, the storage of glycosaminoglycan (GAG) is due to impaired degradation. This study examines the kinetics of GAG accumulation in Marfan's disease in order to determine whether the mechanism of accumulation differs from that in Hurler's disease.Marfan-derived fibroblasts incorporated [(14)C]acetate or [(14)C]glucosamine into GAG to a level 4-6 times greater than control fibroblasts. Sugar analyses, electrophoretic mobility, and enzyme susceptibility studies showed that the isolated material was hyaluronic acid. There were no differences in activity of a variety of glycosidases between Marfan and control fibroblasts, nor were there differences in the ability to degrade prelabeled hyaluronate by cell-free extracts. Finally, chase experiments showed parallel rates of loss of labeled GAG from control fibroblasts and fibroblasts from Marfan patients. It appears that hyaluronic acid was accumulating in greater amounts in the fibroblasts from patients with Marfan's disease because of a greater rate of synthesis as opposed to a decreased rate of breakdown.
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    • Accession Number:
      0 (Acetates)
      0 (Carbon Isotopes)
      0 (Glycosaminoglycans)
      9004-61-9 (Hyaluronic Acid)
      98600C0908 (Cycloheximide)
      CUB7JI0JV3 (Cetylpyridinium)
      EC 3.2.1.- (Glycoside Hydrolases)
      N08U5BOQ1K (Glucosamine)
    • Publication Date:
      Date Created: 19731001 Date Completed: 19731106 Latest Revision: 20181113
    • Publication Date:
      20221208
    • Accession Number:
      PMC302501
    • Accession Number:
      10.1172/JCI107433
    • Accession Number:
      4125715