title: The chemical composition of tooth enamel in junctional epidermolysis bullosa.
abstract: The junctionalis form of epidermolysis bullosa (EBJ) is associated with a number of clinical problems involving tooth enamel, including increased susceptibility to caries. The aim here was to carry out a chemical characterization of the enamel of teeth from EBJ patients compared with that of unaffected controls. The results showed that while protein concentration, amino acid composition and carbonate content were similar in both groups, EBJ enamel contained a significantly reduced mineral per volume content, resulting in enamel hypoplasia. In addition, Western blotting revealed the presence of serum albumin (a known inhibitor of enamel crystal growth) in EBJ enamel. This was not detected in control enamel or in enamel of teeth from patients with the dystrophic form of the disease. It is concluded that EBJ enamel is developmentally compromised and that the enamel defects are commensurate with the reported genetic lesions.
authors.affiliation: Division of Oral Biology, Leeds Dental Institute, Clarendon Way, Leeds, UK. orl.6jen@oralbio.novell.leeds.ac.uk
authors.forename: J
authors.initials: J
authors.lastname: Kirkham
authors.forename: C
authors.initials: C
authors.lastname: Robinson
authors.forename: S M
authors.initials: SM
authors.lastname: Strafford
authors.forename: R C
authors.initials: RC
authors.lastname: Shore
authors.forename: W A
authors.initials: WA
authors.lastname: Bonass
authors.forename: S J
authors.initials: SJ
authors.lastname: Brookes
authors.forename: J T
authors.initials: JT
authors.lastname: Wright
mesh_terms: D000566:Amelogenesis; D000596:Amino Acids / Q000032:analysis; D015153:Blotting, Western; D002254:Carbonates / Q000032:analysis; D002648:Child; D003460:Crystallization; D003733:Dental Caries Susceptibility; D003743:Dental Enamel / Q000737:chemistry*; D003744:Dental Enamel Hypoplasia / Q000378:metabolism; D003746:Dental Enamel Proteins / Q000032:analysis; D016108:Epidermolysis Bullosa Dystrophica / Q000378:metabolism; D016109:Epidermolysis Bullosa, Junctional / Q000378:metabolism*; D006801:Humans; D008903:Minerals / Q000032:analysis; D012709:Serum Albumin / Q000032:analysis
chemical_list: D000596:Amino Acids; D002254:Carbonates; D003746:Dental Enamel Proteins; D008903:Minerals; D012709:Serum Albumin