Hereditary dentine disorders are divided into 5 types: 3 types of dentinogenesis imperfecta (DGI), and 2 types of dentin dysplasia (DTDP). DGI type I is inherited with osteogenesis imperfecta [DS:H00506]. It has been shown that mutations in the genes encoding collagen type 1, COL1A1 and COL1A2, underlie this condition. All other forms of DGI and DTDP except DTDP1, appear to result from mutations in the gene encoding dentine sialophosphoprotein (DSPP).
Category
Congenital malformation
Brite
Human diseases in ICD-11 classification [BR:br08403]
20 Developmental anomalies
Structural developmental anomalies primarily affecting one body system
Structural developmental anomalies of the face, mouth or teeth
LA30 Structural developmental anomalies of teeth and periodontal tissues
H00432 Hereditary dentine disorders
Pathway-based classification of diseases [BR:br08402]
Cellular process
nt06548 Integrin signaling
H00432 Hereditary dentine disorders
