A genetic anomaly of oriented collagen biosynthesis and cross-linking: Keratoconus.

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  • Additional Information
    • Source:
      Publisher: Elsevier Country of Publication: France NLM ID: 0265365 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1768-3114 (Electronic) Linking ISSN: 03698114 NLM ISO Abbreviation: Pathol Biol (Paris) Subsets: MEDLINE
    • Publication Information:
      Publication: 2000- : Paris : Elsevier
      Original Publication: Paris, Expansion scientifique française.
    • Subject Terms:
      Protein Multimerization*/genetics; Collagen/*biosynthesis ; Collagen/*chemistry; Collagen/genetics ; Cornea/metabolism ; Cornea/pathology ; Extracellular Matrix/metabolism ; Extracellular Matrix/pathology ; Extracellular Space/metabolism ; Humans ; Keratoconus/genetics ; Keratoconus/metabolism ; Keratoconus/pathology ; Proteoglycans/metabolism
    • Abstract:
      Oriented collagen biosynthesis is one of the major mechanisms involved in tissue and organ formation during development. Corneal biogenesis is one example. Defects in this process lead to anomalies in tissue structure and function. The transparency of cornea and its achievement are a good example as well as its pathological modifications. Keratoconus is one example of this type of pathologies, involving also inappropriate cross-linking of collagen fibers. Among the tentatives to correct this anomaly, the riboflavin-potentiated UV-cross-linking (CXL) of keratoconus corneas appears clinically satisfactory, although none of the experiments and clinical results published prove effective cross-linking. The published results are reviewed in this article.
      (Copyright © 2014. Published by Elsevier SAS.)
    • Contributed Indexing:
      Keywords: CXL; Collagen; Collagène; Cornea; Cornée; Cross-linking; Keratoconus; Kératocône; Pontage; Riboflavin-UV-mediation; Stimulation par riboflavine et UV
    • Accession Number:
      0 (Proteoglycans)
      9007-34-5 (Collagen)
    • Publication Date:
      Date Created: 20141204 Date Completed: 20160829 Latest Revision: 20150209
    • Publication Date:
      20240628
    • Accession Number:
      10.1016/j.patbio.2014.10.004
    • Accession Number:
      25468492