title: A genetic anomaly of oriented collagen biosynthesis and cross-linking: Keratoconus.
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.
authors.affiliation: Centre d'ophtalmologie HUPC, hôpital Hôtel-Dieu, Assistance publique-Hôpitaux de Paris, 1, place du Parvis-Notre-Dame, 75181 Paris cedex 04, France; Faculté de médicine Paris Descartes, université Sorbonne Paris Cité, 15, rue de l'École-de-Médecine, 75006 Paris, France.
authors.forename: J L
authors.initials: JL
authors.lastname: Bourges
authors.affiliation: Faculté de médicine Paris Descartes, université Sorbonne Paris Cité, 15, rue de l'École-de-Médecine, 75006 Paris, France; Laboratoire de recherche ophtalmologique, hôpital Hôtel-Dieu, Assistance publique-Hôpitaux de Paris, 1, place du Parvis-Notre-Dame, 75181 Paris cedex 04, France.
authors.forename: A M
authors.initials: AM
authors.lastname: Robert
authors.affiliation: Faculté de médicine Paris Descartes, université Sorbonne Paris Cité, 15, rue de l'École-de-Médecine, 75006 Paris, France; Laboratoire de recherche ophtalmologique, hôpital Hôtel-Dieu, Assistance publique-Hôpitaux de Paris, 1, place du Parvis-Notre-Dame, 75181 Paris cedex 04, France. Electronic address: lrobert5@orange.fr.
authors.forename: L
authors.initials: L
authors.lastname: Robert
mesh_terms: D003094:Collagen / Q000096:biosynthesis* / Q000737:chemistry* / Q000235:genetics; D003315:Cornea / Q000378:metabolism / Q000473:pathology; D005109:Extracellular Matrix / Q000378:metabolism / Q000473:pathology; D005110:Extracellular Space / Q000378:metabolism; D006801:Humans; D007640:Keratoconus / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D055503:Protein Multimerization / Q000235:genetics; D011509:Proteoglycans / Q000378:metabolism
chemical_list: D011509:Proteoglycans; D003094:Collagen
keywords: CXL; Collagen; Collagène; Cornea; Cornée; Cross-linking; Keratoconus; Kératocône; Pontage; Riboflavin-UV-mediation; Stimulation par riboflavine et UV