title: [Lysosomal storage diseases, genetic or drug-induced? effect of glycosaminoglycan and sphingolipid disorders on dental tissues].
abstract: In vivo studies were carried out on dental tissues of rat incisor after a single injection of suramin, a drug which induces mucopolysaccharidosis-like disease. Accumulation of lysosome-like structures was seen in secretory ameloblasts and odontoblasts. In vitro studies on embryonic tooth germ buds showed similar changes when they were cultured in presence of suramin. Anti-phospholipid immunolabelling revealed a developmentally regulated temporo-spatial pattern. Radiolabeling with 3H-suramin indicated cytosolic and nuclear incorporation. The drug acting as polyanion interacted directly with predentine. 35S sulphate incorporation was impaired by the drug. Another lysosomal storage disease, the sphingolipidosis, Krabbe's disease was also investigated in human. Changes were observed in pulp cells and as a consequence in dentin. Enamel also displayed many changes. Pharmacological or genetically acquired diseases constitute models providing insights on the role played by glycosaminoglycans and phospholipids in biomineralization.
authors.affiliation: Laboratoire de Biologie et Biomatériaux du Milieu Buccal et Osseux, Faculté de Chirurgie Dentaire, Paris V, Montrouge, France.
authors.forename: M
authors.initials: M
authors.lastname: Goldberg
authors.forename: A
authors.initials: A
authors.lastname: Gritli
authors.forename: A
authors.initials: A
authors.lastname: Bloch-Zupan
authors.forename: D
authors.initials: D
authors.lastname: Septier
authors.forename: S
authors.initials: S
authors.lastname: Lécolle
authors.forename: J M
authors.initials: JM
authors.lastname: Legrand
authors.forename: J V
authors.initials: JV
authors.lastname: Ruch
mesh_terms: D000565:Ameloblasts / Q000473:pathology; D000818:Animals; D002648:Child; D003804:Dentin / Q000473:pathology*; D006025:Glycosaminoglycans / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D007965:Leukodystrophy, Globoid Cell / Q000473:pathology*; D051379:Mice; D009083:Mucopolysaccharidoses / Q000139:chemically induced* / Q000473:pathology; D009804:Odontoblasts / Q000473:pathology; D051381:Rats; D013107:Sphingolipids / Q000378:metabolism*; D013498:Suramin / Q000494:pharmacology; D014094:Tooth, Deciduous / Q000473:pathology
chemical_list: D006025:Glycosaminoglycans; D013107:Sphingolipids; D013498:Suramin
vernacular_title: Maladies de surcharge lysosomale, génétiques ou induites pharmacologiquement: effets de pathologies de glycosaminoglycanes et de sphingolipides sur les tissus dentaires.