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2007 ; 186
(1
): 70-7
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Disorders of human dentin
#MMPMID17627120
Hart PS
; Hart TC
Cells Tissues Organs
2007[]; 186
(1
): 70-7
PMID17627120
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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.