A Dentin Sialophosphoprotein Mutation That Partially Disrupts a Splice Acceptor Site Causes Type II Dentin Dysplasia
نویسندگان
چکیده
منابع مشابه
Developmental Disturbance of Dentin with Probable Diagnosis of Dentin Dysplasia Type 1d: A rare case report
Background: Dentin dysplasia (DD) is a rare disturbance of dentin formation, characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology. In DD type I, the teeth appear clinically normal in morphologic appearance and color. Radiographic analysis shows obliteration of all pulp chambers as well as short, blunted, and malformed or absent roots with multiple periapi...
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A case report on dentin dysplasia type I, a congenital disease (autosomal dominant gene defect) affecting deciduous and permanent teeth, is depicted including representations of clinical and histological features, X-ray and CT-findings. Therapy includes extraction of all teeth, ectomization of cystic alteration, revision of paranasal sinus. Aesthetic and functional rehabilitation by means of in...
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Introduction: Dentin Dysplasia is one of the rare hereditary diseases that the enamel is normal, but it also affects the dentin and the shape of the tooth pulp. Due to the low prevalence of this disease, a case of dentin dysplasia with the classical and atypical dentin dysplasia type 1 is presented. Case presentation:A 11-year-old girl with the mobility of lower central teeth that has severe ...
متن کاملMolecular cloning of a human dentin sialophosphoprotein gene.
Dentin sialoprotein (DSP) and dentin phosphoprotein (DPP; phosphophoryn) are two principal dentin-specific non-collagenous proteins. DPP is extremely acidic and is rich in aspartic acid and serine. By virtue of this structure, DPP may bind large amounts of calcium and may facilitate initial mineralization of dentin matrix collagen as well as regulate the size and shape of the crystals. The func...
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Introduction: Pathogenic variants of RUNX2, a gene that encodes an osteoblast-specific transcription factor, have been shown as the cause of CCD, which is a rare hereditary skeletal and dental disorder with dominant mode of inheritance and a broad range of clinical variability. Due to the relative lack of clinical complications resulting in CCD, the medical diagnosis of this disorder is challen...
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ژورنال
عنوان ژورنال: Journal of Endodontics
سال: 2008
ISSN: 0099-2399
DOI: 10.1016/j.joen.2008.08.027