Hereditary Dentin Defects

Dan Boboia 2/5/10 Lit. Review

Title: Hereditary Dentin Defects
Author: Kim et al
Type of Article: Review

Main Purpose:
To discuss the development of the dentin extracellular matrix in the context of it’s evolution, and discuss the phenotypes and clinical classifications of isolated hereditary defects of tooth dentin in the context of recent genetic data respecting their genetic etiologies.

Summary:
Inherited defects are divided into 5 types: 3 types of DGI (DGY types 1, 2, and 3), and 2 types of dentinal dysplasia (DD-1 and DD-2). DGI type 1 is OI with DGI. This is caused by mutations in the two genes encoding type I collagen. DD-II, DGI-II, DGI-III all have their own pattern of inherited defects limited to the dentition. DD-I has an etiology which is still a mystery. It features short, blunt roots with obliterated pulp chambers and abnormal crowns (color, shape, and form). A major surprise in the characterization of genes underlying inherited dentin defects is the lack of roles played by genes encoding less-abundant non-collagenous proteins in dentin, such as DMP1, IBSP, MEPE, SPP1, and OPN.

Evaluation:
A very dense article which requires multiple readings in order to “digest” all of the genetic material presented. Can anyone say Braz Macedo.