epidermolysis bullosa (eb) is an inherited disorder, which is characteristically presented as skin blisters developing in response to minor injury. junctional variety of eb is also associated with enamel hypoplasia. amelogenesis imperfecta presents with abnormal formation of the enamel both in deciduous and permanent dentition. this article describes a previously unreported case of amelogenesis...

Introduction: Dentin dysplasia is a rare autosomal dominant inheriting disturbance of dentin formation characterized by normal enamel formation, but atypical dentin with abnormal pulpal morphology. There are two major patterns: type I and type II. Amelogenesis imperfecta is an autosomal dominant. X-link inherent disease that is classified by clinical manifestation into hypoplastic, hyp...

Ameloblasts produce enamel matrix proteins such as amelogenin, ameloblastin, and amelotin during tooth development. The molecular mechanisms of ameloblast differentiation (amelogenesis) are currently not well understood. SP6 is a transcription factor of the Sp/KLF family that was recently found to regulate cell proliferation in a cell-type-specific manner. Sp6-deficient mice demonstrate charact...

Amelogenesis is a tightly regulated and intricate process of enamel formation. It involves epithelial-mesenchymal interactions at the embryological level by means of enamel proteins expression. During this process, ameloblasts undergo morphological changes which correspond to the pre-synthetic, synthetic and post-synthetic phases. Regulatory changes within these cells have been studied at light...

Amelogenesis imperfecta has been defined as a group of hereditary enamel defects which may or may not be associated with some other dental and skeletal developmental defects. It can be characterized by enamel hypoplasia, hypomaturation, or hypo-calcification of the teeth. This clinical report describes the oral rehabilitation of a twenty two year old female patient with amelogenesis imperfecta ...

Numerous cases of enamel renal syndrome have been previously reported. Various terms, such as enamel renal syndrome, amelogenesis imperfecta and gingival fibromatosis syndrome, and enamel-renal-gingival syndrome, have been used for patients presenting with the dental phenotype characteristic of this condition, nephrocalcinosis or nephrolithiasis, and gingival findings. This report describes a c...

Teeth fragments from members of a family clinically and genetically diagnosed as having amelogenesis imperfecta were studied by scanning electron microscopy and X-ray microprobe analysis to establish the morphological patterns and the quantitative concentration of calcium in the enamel of anterior (canine, incisor) and posterior (premolar and molar) teeth. The prism patterns in the enamel of te...

introduction: dentin dysplasia is a rare autosomal dominant inheriting disturbance of dentin formation characterized by normal enamel formation, but atypical dentin with abnormal pulpal morphology. there are two major patterns: type i and type ii. amelogenesis imperfecta is an autosomal dominant. x-link inherent disease that is classified by clinical manifestation into hypoplastic, hypomature, ...

amelogenesis imperfecta is a heterogeneous group of hereditary disorders. its treatment continues throughout the patients' childhood and adolescence and consists of advanced restorative care in severe cases. a thorough prosthodontic treatment plan including orthognatic surgery, full veneer crowns and all ceramic anterior crowns is presented in this clinical report.

Syndecans are transmembranous heparan sulfate proteoglycans (HSPGs) with covalently attached glycosaminoglycan side-chains located on the cell surface. The mammalian syndecan family is composed of four types of syndecans (syndecan-1 to -4). Syndecans interact with the intracellular cytoskeleton through the cytoplasmic domains of their core proteins and membrane proteins, extracellular enzymes, ...