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Disorders of Human Dentin

Hart P.S.a · Hart T.C.b

Author affiliations

aNational Human Genome Research Institute, and bSection of Dental and Craniofacial Genetics, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Md., USA

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Cells Tissues Organs 2007;186:70–77

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Article / Publication Details

First-Page Preview
Abstract of Paper

Received: December 20, 2006
Accepted: December 20, 2006
Published online: July 06, 2007
Issue release date: July 2007

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 2

ISSN: 1422-6405 (Print)
eISSN: 1422-6421 (Online)

For additional information: https://www.karger.com/CTO

Abstract

Dentin, the most abundant tissue in teeth, is produced by odontoblasts, which differentiate from mesenchymal cells of the dental papilla. Dentinogenesis is a highly controlled process that results in the conversion of unmineralized predentin to mineralized dentin. By weight, 70% of the dentin matrix is mineralized, while the organic phase accounts for 20% and water constitutes the remaining 10%. Type I collagen is the primary component (>85%) of the organic portion of dentin. The non-collagenous part of the organic matrix is composed of various proteins, with dentin phosphoprotein predominating, accounting for about 50% of the non-collagenous part. Dentin defects are broadly classified into two major types: dentinogenesis imperfectas (DIs, types I–III) and dentin dysplasias (DDs, types I and II). To date, mutations in DSPP have been found to underlie the dentin disorders DI types II and III and DD type II. With the elucidation of the underlying genetic mechanisms has come the realization that the clinical characteristics associated with DSPP mutations appear to represent a continuum of phenotypes. Thus, these disorders should likely be called DSPP-associated dentin defects, with DD type II representing the mild end of the phenotypic spectrum and DI type III representing the severe end.

© 2007 S. Karger AG, Basel


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Article / Publication Details

First-Page Preview
Abstract of Paper

Received: December 20, 2006
Accepted: December 20, 2006
Published online: July 06, 2007
Issue release date: July 2007

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 2

ISSN: 1422-6405 (Print)
eISSN: 1422-6421 (Online)

For additional information: https://www.karger.com/CTO


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