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DGI3
MCID: DNT044
MIFTS: 26
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Dentinogenesis Imperfecta, Shields Type Iii (DGI3)
Categories:
Genetic diseases, Oral diseases, Rare diseases
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MalaCards integrated aliases for Dentinogenesis Imperfecta, Shields Type Iii:
Characteristics:Orphanet epidemiological data:58
dentinogenesis imperfecta type 3
Inheritance: Autosomal dominant; Age of onset: Childhood; OMIM®:57 (Updated 20-May-2021)
Inheritance:
autosomal dominant
Miscellaneous:
shields classification - type 1 - associated with osteogenesis imperfecta type 2 - hereditary opalescent dentin, not associated with bone defect type 3 - brandywine isolate opalescent dentin occurs in a southern maryland tri-racial inbred population known as the "brandywine isolate" HPO:31Classifications:
ICD10:
33
Orphanet: 58
Rare odontological diseases |
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GARD :
20
Dentinogenesis imperfecta type 3 is a rare and severe form of dentinogenesis imperfecta, a condition that affects tooth development. People affected by this condition generally have discolored (most often a blue-gray or yellow-brown color) and translucent teeth. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. Dentinogenesis imperfecta type 3 is caused by changes ( mutations ) in the DSPP gene which are inherited in an autosomal dominant manner. Treatment is usually focused on protecting primary (baby) and then permanent teeth with preformed pediatric crowns and other interventions. The replacement of teeth might be considered in the future with dentures and/or implants.
MalaCards based summary : Dentinogenesis Imperfecta, Shields Type Iii, also known as brandywine type dentinogenesis imperfecta, is related to dentinogenesis imperfecta and dentinogenesis imperfecta 1. An important gene associated with Dentinogenesis Imperfecta, Shields Type Iii is DSPP (Dentin Sialophosphoprotein). Affiliated tissues include bone, and related phenotypes are dentinogenesis imperfecta and anterior open-bite malocclusion UniProtKB/Swiss-Prot : 72 Dentinogenesis imperfecta, Shields type 3: 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.
More information from OMIM:
125500
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Diseases related to Dentinogenesis Imperfecta, Shields Type Iii via text searches within MalaCards or GeneCards Suite gene sharing:
Graphical network of the top 20 diseases related to Dentinogenesis Imperfecta, Shields Type Iii:
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Human phenotypes related to Dentinogenesis Imperfecta, Shields Type Iii:31
Symptoms via clinical synopsis from OMIM®:57 (Updated 20-May-2021)Clinical features from OMIM®:125500 (Updated 20-May-2021) |
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MalaCards organs/tissues related to Dentinogenesis Imperfecta, Shields Type Iii:40
Bone
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Articles related to Dentinogenesis Imperfecta, Shields Type Iii:(show all 23)
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Genes related to Dentinogenesis Imperfecta, Shields Type Iii (1 elite genes): - Elite gene
- Cancer Census gene in COSMIC
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ClinVar genetic disease variations for Dentinogenesis Imperfecta, Shields Type Iii:6
UniProtKB/Swiss-Prot genetic disease variations for Dentinogenesis Imperfecta, Shields Type Iii:72
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Search
GEO
for disease gene expression data for Dentinogenesis Imperfecta, Shields Type Iii.
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