Amelogenesis imperfecta (AI) presents with a rare abnormal formation of the enamel or external layer of the crown of teeth. Enamel is composed mostly of mineral, that is formed and regulated by the proteins in it. Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel: ameloblastin, enamelin, tuftelin and amelogenin.|(ICD-10 K00.5)|Hereditary disturbances in tooth structure, not elsewhere classified.