Dentine dysplasia type II is an autosomal dominant disorder in which mineralization of the dentine of the primary teeth is abnormal. On the basis of the phenotypic overlap between, and shared chromosomal location with, dentinogenesis imperfecta type II, a second disorder of dentine mineralization, it has been proposed that the two conditions are allelic. As recent studies have shown that dentin...

Dentin sialophosphoprotein (Dspp) is mainly expressed in teeth by the odontoblasts and preameloblasts. The Dspp mRNA is translated into a single protein, Dspp, and cleaved into two peptides, dentin sialoprotein and dentin phosphoprotein, that are localized within the dentin matrix. Recently, mutations in this gene were identified in human dentinogenesis imperfecta II (Online Mendelian Inheritan...

The aim of this study was to determine the prevalence and incidence evaluate current status dental treatment Amelogenesis imperfecta (AI) Dentinogenesis (DI) in South Korea. data based on National Health Insurance Service (NHIS)-National Sample Cohort Database (2002 - 2015) Jeonbuk University (JBNU) Dental Hospital (2011 2020). NHIS analysis showed AI DI were 11.6 2.4 per 100,000 people, respec...

Dental hard tissue is subject to variety of disorders. Dentinogenesis Imperfecta is one such disorder attributed to heredity. It is known to be an autosomal dominant trait. Teeth with such 'imperfect' dentin are liable to be weak and discolored. The disease has variable penetration and therefore can be expressed as a range of phenotypic manifestations from mild discoloration and chipping to fra...

Osteogenesis imperfecta (OI), also known as "brittle bone disease", is a rare genetic disorder of the skeleton, whose most benign form I corresponds to autosomal dominant mutations in genes encoding type collagen (COLA1, COLA2). Several associated skeletal manifestations are often observed but, surprisingly, while dentin defects reflect disorders, about half OI patients have no obvious oral man...

Osteogenesis Imperfecta (OI) is a heterogeneous genetic disorder, with features that include increased bone fragility, pathological fractures, blue sclera, dentinogenesis imperfecta and conductive or mixed hearing loss. Clinical variability is wide from children with few fractures and normal stature to children with multiple fractures, long bone deformity, scoliosis and extreme short stature. A...