Amelogenesis Imperfecta (AI) is a clinical diagnosis that encompasses a group of genetic mutations, each affecting processes involved in tooth enamel formation and thus, result in various enamel defects. The hypomaturation enamel phenotype has been described for mutations involved in the later stage of enamel formation, including Klk4, Mmp20, C4orf26, and Wdr72. Using a candidate gene approach ...

Developmental defects of the enamel (D.D.E.) are changes in the deciduous dentition that have been little studied in Brazil, although they lead to aesthetic problems, dental sensitivity and may be predictors of dental caries. The objective of this study was to estimate the prevalence and distribution of D.D.E. in the deciduous dentition of pre-school children in the municipality of Itajaí, Sant...

Mutations in the human enamelin gene cause autosomal dominant hypoplastic amelogenesis imperfecta in which the affected enamel is thin or absent. Study of enamelin knockout NLS-lacZ knockin mice revealed that mineralization along the distal membrane of ameloblast is deficient, resulting in no true enamel formation. To determine the function of enamelin during enamel formation, we characterized ...

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...

Amelogenesis imperfecta (AI) describes a broad group of clinically and genetically heterogeneous inherited defects of dental enamel bio-mineralization. Despite identification of a number of genetic mutations underlying AI, the precise causal mechanisms have yet to be determined. Using a multi-disciplinary approach, we describe here a mis-sense mutation in the mouse Amelx gene resulting in a Y -...

Amelogenesis imperfecta (AI) designates a group of genetic diseases characterized by a large range of enamel disorders causing important social and health problems. These defects can result from mutations in enamel matrix proteins or protease encoding genes. A range of mutations in the enamel cleavage enzyme matrix metalloproteinase-20 gene (MMP20) produce enamel defects of varying severity. To...

This study examines the inter-relation between enamel morphology and crack resistance by sectioning extracted human molars after loading to fracture. Cracks appear to initiate from tufts, hypocalcified defects at the enamel-dentin junction, and grow longitudinally around the enamel coat to produce failure. Microindentation corner cracks placed next to the tufts in the sections deflect along the...

Enamel is one of the most important structures of the tooth, both from a functional and esthetic point of view. Primary enamel carries registered information regarding metabolic and physiological events that occurred during the period around birth and the first year of life. Detailed knowledge of normal development and the structure of enamel is important for the assessment of mineralization de...

Hypomineralized enamel defects frequently are manifest as a mottled-white appearance and can be associated with variable degrees of discrete yellow-brown intrinsic staining. Numerous treatment approaches have been proposed, ranging from bleaching to enamel reduction to restorative techniques. Bleaching of hypomineralized enamel lesions, using 1 to 2 applications (10 to 15 minutes each) of 5% so...