You are using an outdated browser. Please upgrade your browser to improve your experience.

dentinogenesis imperfecta type 3

Disease Summary
Associated Targets (1)
Tbio

1


GARD Rare
Mondo Description Dentinogenesis imperfecta type 3 (DGI-3) is a rare, severe form of dentinogenesis imperfecta (DGI) characterized by opalescent primary and permanent teeth, marked attrition, large pulp chambers, multiple pulp exposure and shell teeth radiographically (i.e. teeth which appear hollow due to dentin hypotrophy).
Uniprot Description A form of dentinogenesis imperfecta, an autosomal dominant dentin disorder characterized by amber-brown, opalescent teeth that fracture and shed their enamel during mastication, thereby exposing the dentin to rapid wear. Radiographically, the crown appears bulbous and pulpal obliteration is common. The pulp chambers are initially larger than normal prior and immediately after tooth eruption, and then progressively close down to become almost obliterated by abnormal dentin formation. Roots are short and thin. Both primary and permanent teeth are affected. DGI3 teeth typically manifest multiple periapical radiolucencies. DGI3 is not associated with osteogenesis imperfecta.
Mondo Term and Equivalent IDs
MONDO:0007442:  dentinogenesis imperfecta type 3
GARD:0010144: 
MESH:C538216: 
Orphanet:166265: 
SCTID:234970006: