dentin dysplasia/lamyropsis microcephala

According to their phenotypic features, the hereditary dentin defects in humans are categorized in two major classes: dentinogenesis imperfecta and dentin dysplasia. At its turn, the dentin dysplasia is subdivided in dentin dysplasia type I and dentin dysplasia type II, a milder clinical

The dentin sialophosphoprotein (DSPP) gene on chromosome 4q21.3 encodes the major noncollagenous protein in tooth dentin. DSPP mutations are the principal cause of dentin dysplasia type II, dentinogenesis imperfecta type II, and dentinogenesis imperfecta type III. We have identified a DSPP splice

Dentin dysplasia, type II (MIM*125420) is an autosomal dominant disorder of dentin development. Clinically the primary dentition appears opalescent, and radiographically the pulp chambers are obliterated, resembling dentinogenesis imperfecta. However, unlike dentinogenesis imperfecta, the permanent

Dentin dysplasia, Type II, is a rare autosomal dominant disorder. The primary teeth are amber and translucent and the pulp chambers are obliterated. The permanent teeth have a normal to brown-gray coloration and a thistle-tube pulp configuration with multiple true denticles. To date, only five

Dentin dysplasia, type II, is an inherited autosomal dominant disorder in which primary teeth are amber and translucent, with pulp chambers obliterated by abnormal dentin. The permanent teeth have a normal coronal morphologic character and coloration but exhibit "thistle tube"-shaped pulp chambers

Dentinogenesis imperfecta (DGI) and dentin dysplasia (DD) are allelic disorders that primarily affect the formation of tooth dentin. Both conditions are autosomal-dominant and can be caused by mutations in the dentin sialophosphoprotein gene (DSPP, 4q21.3). We recruited 23 members of a