Change search
ReferencesLink to record
Permanent link

Direct link
The Structure and Composition of Deciduous Enamel Affected by Local Hypoplastic Autosomal Dominant Amelogenesis Imperfecta Resulting from an ENAM Mutation
Leeds Dental Institute, University of Leeds, Leeds, UK.
Umeå University, Faculty of Medicine, Department of Odontology, Pediatric Dentistry.
School of Clinical Dentistry, University of Sheffield, Sheffield, UK.
School of Dental Sciences, University of Liverpool, Liverpool, UK.
Show others and affiliations
2010 (English)In: Cells Tissues Organs, ISSN 1422-6405, E-ISSN 1422-6421, Vol. 191, no 4, 301-306 p.Article in journal (Refereed) Published
Abstract [en]

In a group of families in northern Sweden, a mutation in the ENAM gene (predicted to produce a highly truncated protein) results in the local hypoplastic form of autosomal dominant amelogenesis imperfecta. In this study, sections of deciduous teeth from members of 3 of these families were examined by scanning electron microscopy (SEM) and the enamel mineral was analysed by energy dispersive X-ray spectroscopy (EDX). The sections were also probed with antibodies raised to a conserved sequence of the enamelin protein. Selected intact teeth were first analysed by digital imaging and ascribed with an 'Enamel Defects Index' (EDI) score. SEM of tooth sections revealed disrupted prism morphology and the prisms had a glass-like appearance in some areas. These areas of dysplasia were sometimes irregular but formed regular arrays in others. Comparison of EDI scores with SEM indicated that in one tooth the surface had no measurable defects but significant defects were present in the underlying enamel microstructure. SEM immunohistochemistry with the antibody raised to a fragment of the enamelin protein produced positive, but light, labelling throughout normal enamel. In dysplastic areas, however, the labelling intensity appeared to be reduced. The results indicate that the presence of functional enamelin in the correct amounts is necessary for correct prism morphogenesis. In addition, a combination of EDI and structural analysis indicate that defects in enamel microstructure are not necessarily visible as defects on the surface of the tooth, suggesting the possibility, at least, that some instances of under-diagnosis may occur. Copyright (C) 2009 S. Karger AG, Basel

Place, publisher, year, edition, pages
S. Karger, 2010. Vol. 191, no 4, 301-306 p.
Keyword [en]
Enamel, Amelogenesis imperfecta, Local hypoplastic, ENAM, Enamelin
National Category
URN: urn:nbn:se:umu:diva-109588DOI: 10.1159/000258703ISI: 000275679800004PubMedID: 19923784OAI: diva2:858535
Available from: 2015-10-02 Created: 2015-10-01 Last updated: 2015-10-02Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Bäckman, B.
By organisation
Pediatric Dentistry
In the same journal
Cells Tissues Organs

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 16 hits
ReferencesLink to record
Permanent link

Direct link