Amelogenesis Imperfecta is a Disorder that Causes a Lack of Protein in Teeth

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Amelogenesis Imperfecta is a tooth development disorder which is caused by lack of protein in the tooth enamel. Amelogenesis imperfecta affects the dental enamel, occasionally in conjunction with other dental, oral and extraoral tissues (Crawford). This disorder causes teeth to be discolored, unusually small, and inclined to rapid wear and breakage (“Amelogenesis Imperfecta”). Researchers have described at least fourteen forms of amelogenesis imperfecta which can vary among affected individuals of both primary teeth and permanent teeth (“Amelogenesis Imperfecta”). The essential genes for normal tooth development which provide instructions for making proteins in the enamel are the AMELX, ENAM, and MMPO20 genes (Office of Rare Diseases Research). Proteins such as amelogenin, ameloblastin, and enamelin are critical for normal formation of the enamel (“Amelogenesis Imperfecta”). Enamel is the protective layer of tissue which protects the tooth from painful temperatures end chemicals (Office of Rare Diseases Research). “Enamel is 97% mineral by weight with approximately 1% protein and 2% water” (Wright). In Amelogenesis Imperfecta, the AMELX, ENAM, and MMP20 genes will be mutated and will alter the structure of the proteins or prevent any proteins from being made at all (“Amelogenesis Imperfecta”). This condition presents problems of socializing with others and discomfort, but they may be managed early by vigorous
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