dentinogenesis imperfecta

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dentinogenesis

 [den″tĭ-no-jen´ĕ-sis]
the formation of dentin.
dentinogenesis imperfec´ta a hereditary disorder of tooth development, transmitted as an autosomal dominant trait, and characterized by discoloration of the teeth, ranging from dusky blue to brownish, poorly formed dentin with an abnormally low mineral content, obliteration of the root canal, and normal enamel. The teeth usually wear down rapidly, leaving short, brown stumps.

den·tin·o·gen·e·sis im·per·fec·ta

[MIM*125490 & MIM*125500]
an autosomal dominant disorder of the teeth characterized clinically by translucent gray to yellow-brown teeth involving both primary and permanent dentition; the enamel fractures easily, leaving exposed dentin, which undergoes rapid attrition; radiographically, the pulp chambers and canals appear obliterated and the roots are short and blunted; sometimes occurs in association with osteogenesis imperfecta; autosomal dominant inheritance.

dentinogenesis imperfecta

A common (1 in 6–8,000) inherited disorder of tooth development (OMIM:125490), which is characterised by blue-gray or yellow-brown translucencies of the teeth.

Molecular pathology
Dentinogenesis imperfecta is caused by mutations of DSPP, the gene that encodes two key proteins of the dentin extracellular matrix: dentin sialoprotein and dentin phosphoprotein.

den·tin·o·gen·e·sis im·per·fec·ta

(den'ti-nō-jen'ĕ-sis im-pĕr-fek'tă)
A hereditary disorder of the teeth characterized by translucent gray to yellow-brown teeth involving both primary and permanent dentition; the enamel fractures easily, leaving exposed dentine that undergoes rapid attrition; radiographically, the pulp chambers and canals appear obliterated and the roots are short and blunted; sometimes occurs in association with osteogenesis imperfecta.

den·tin·o·gen·e·sis im·per·fec·ta

(den'ti-nō-jen'ĕ-sis im-pĕr-fek'tă) [MIM*125490 & 125500, MIM*125490, MIM*125500, MIM*125490 &]
Dental disorder characterized clinically by translucent gray to yellow-brown teeth involving both primary and permanent dentition; such enamel fractures easily, leaving exposed dentin, which undergoes rapid attrition.
Synonym(s): hereditary opalescent dentin (1) .