Dentin sialophosphoprotein (DSPP) mutations cause dentin dysplasia type II (DD-II) and dentinogenesis imperfecta types II and III (DGI-II and DGI-III, respectively). We identified two kindreds with DGI-II who exhibited vertical bands of hypoplastic enamel. Both families had a previously reported DSPP mutation that segregated with the disease phenotype. Oral photographs and dental radiographs of...

The association of dentinogenesis imperfecta (DI) with a distinct form of chondrodysplasia in a boy was reported by Goldblatt et al. [1991; Am J Med Genet 39:170-172] and has been given the name of Goldblatt syndrome or odontochondrodysplasia (ODCD; OMIM#184260). Since the original description, only four further individuals have been reported (one sib pair and two unrelated cases). We report on...

Mice harboring the Col1a2(oim) mutation (oim) express dentinogenesis imperfecta. To determine the effect of Col1a2 genotype on tissue mechanical properties, we compared Young's modulus and hardness of dentin in the 3 Col1a2 genotypes. Upper incisors were tested by nanoindentation. Genotype had a significant effect on Young's modulus, but there was not a simple mutant allele dosage relationship....

Dentin sialoprotein (DSP) is a major glycoprotein present in the mineralized dentin matrix that is expressed mainly by young and mature odontoblasts. Mutations in the DSP coding regions are linked to Dentinogenesis imperfecta I and II. indicating the importance of DSP in tooth formation. Previous studies have identified multiple mRNA transcripts in dentin that code for both DSP and phosphophory...

The ultimate goal of a regenerative pulp treatment strategy is to reconstitute normal tissue continuum at the pulp-dentin border, regulating tissue-specific processes of tertiary dentinogenesis. Experimental investigations in mature teeth have shown that a network of extracellular matrix molecules and growth factors signal tertiary dentinogenesis. Application of dentin matrix components or grow...

AIM To examine the feasibility of using the pOBCol3.6GFPtpz [3.6-green fluorescent protein (GFP)] transgenic mice as an in vivo model for studying the biological sequence of events during pulp healing and reparative dentinogenesis. METHODOLOGY Pulp exposures were created in the first maxillary molar of 12-16-week-old 3.6-GFP transgenic mice with CD1 and C57/Bl6 genetic background. Direct pulp...

INTRODUCTION This paper has evaluated the dental and or facial disorders associated to Goldblatt syndrome, also known as odontochondrodysplasia. AIM We report the analysis performed on a female young patient affected by this disease. We analyzed her dental and or facial features. MATERIALS AND METHODS We adopted several diagnostic criteria: firstly, we performed radiographic investigations,...

Nonsyndromic dentin defects classified as type II dentin dysplasia and types II and III dentinogenesis imperfecta are caused by mutations in DSPP (dentin sialophosphoprotein). Most reported disease-causing DSPP mutations occur within the repetitive DPP (dentin phosphoprotein) coding sequence. We characterized the DPP sequences of five probands with inherited dentin defects using single molecule...

Osteogenesis imperfecta presents with unique challenges to the anaesthetist. We report a case of osteogenesis imperfecta, posted for rush rodding femur managed caudal anaesthesia and sedation dexmedetomidine ketamine.