Dentin Dysplasia

An apparently hereditary disorder of dentin formation, marked by a normal appearance of coronal dentin associated with pulpal obliteration, faulty root formation, and a tendency for peripheral lesions without obvious cause. (From Dorland, 27th ed)

Also Known As:

Networked: 21 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Dentinogenesis Imperfecta
2.	Cysts
3.	Abscess
4.	Inborn Genetic Diseases (Disease, Hereditary)
5.	Odontodysplasia (Ghost Tooth)

Experts

1.	Hu, Jan C-C: 3 articles (01/2016 - 05/2007)
2.	Simmer, James P: 3 articles (01/2016 - 05/2007)
3.	Kim, Jung-Wook: 2 articles (06/2021 - 12/2008)
4.	Mohan, Karthik Rajaram: 1 article (11/2021)
5.	Narayanan, Mohan: 1 article (11/2021)
6.	Ravikumar, P T: 1 article (11/2021)
7.	Chen, Zhibin: 1 article (09/2021)
8.	Jiang, Yong: 1 article (09/2021)
9.	Jing, Zhaojun: 1 article (09/2021)
10.	Hyun, Hong-Keun: 1 article (06/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Dentin Dysplasia:

1.	Proteins (Proteins, Gene)FDA Link 09/01/2021 - "A large number of previous studies demonstrated that DSPP is a dentinal-specific protein, and DSPP gene mutations lead to dentin dysplasia and dentinogenesis imperfecta. " 01/01/2020 - "Whole-genome RNA-sequencing analysis of embryonic day (E) 14.5 cap stage molars revealed reductions in early expressed enamel matrix components (Odontogenic ameloblast-associated protein) and dentin dysplasia targets (Dentin matrix acidic phosphoprotein 1). "
2.	dentin sialophosphoproteinIBA 01/09/2023 - "Dentin sialophosphoprotein (DSPP) is the pathogenic gene of dentinogenesis imperfecta type Ⅱ, dentinogenesis imperfecta type Ⅲ and dentin dysplasia type Ⅱ. " 01/01/2016 - "Nonsyndromic dentin defects classified as type II dentin dysplasia and types II and III dentinogenesis imperfecta are caused by mutations in DSPP (dentin sialophosphoprotein). " 08/01/2013 - "Dentin dysplasia is a rare autosomal dominant genetic disease characterized by defect of dentin development and the causal gene is DSPP (Dentin Sialophosphoprotein gene). " 06/01/2012 - "Families with nonsyndromic dentinogenesis imperfecta (DGI) and the milder, dentin dysplasia (DD), have mutations in one allele of the dentin sialophosphoprotein (DSPP) gene. " 06/01/2012 - "Rough endoplasmic reticulum trafficking errors by different classes of mutant dentin sialophosphoprotein (DSPP) cause dominant negative effects in both dentinogenesis imperfecta and dentin dysplasia by entrapping normal DSPP."
3.	CollagenIBA 10/01/1992 - "It is suggested that an inherited collagen abnormality in a component common to dentin, skin, ligament and tendon probably explains both EDS I and the dentin dysplasia." 04/06/2001 - "Dentin extracellular matrix components such as type I and III collagens are increased and deposited abnormally in the dental pulp, similar to the hereditary human tooth disorders such as dentin dysplasia and dentinogenesis imperfecta. "
4.	alpha2 Subunit Collagen Type IIBA 05/01/2007 - "Genes expressed by odontoblasts (COL1A1, COL1A2, and DSPP), and ameloblasts (AMELX, ENAM, MMP20, and KLK4) during the crown formation stage, are associated with dentinogenesis imperfecta, dentin dysplasia, and amelogenesis imperfecta. " 06/08/2021 - "Mutations in the gene encoding dentin sialophosphoprotein (DSPP) have been identified to cause dentinogenesis imperfecta (DGI) Types II and III and dentin dysplasia (DD) Type II. While DGI Type I is an OI-related syndromic phenotype caused mostly by monoallelic mutations in the genes encoding collagen type I alpha 1 chain (COL1A1) and collagen type I alpha 2 chain (COL1A2). "
5.	Collagen Type I (Type I Collagen)IBA 06/08/2021 - "Mutations in the gene encoding dentin sialophosphoprotein (DSPP) have been identified to cause dentinogenesis imperfecta (DGI) Types II and III and dentin dysplasia (DD) Type II. While DGI Type I is an OI-related syndromic phenotype caused mostly by monoallelic mutations in the genes encoding collagen type I alpha 1 chain (COL1A1) and collagen type I alpha 2 chain (COL1A2). "
6.	RNA Splice SitesIBA 12/01/2008 - "A dentin sialophosphoprotein mutation that partially disrupts a splice acceptor site causes type II dentin dysplasia."
7.	AmberIBA 02/01/2003 - "Dentinogenesis imperfecta (DI), which is sometimes an accompanying symptom of OI, belongs to a group of genetically conditioned dentin dysplasias and is characterized clinically by an opalescent amber appearance of the dentin. "
8.	RNA (Ribonucleic Acid)IBA 01/01/2020 - "Whole-genome RNA-sequencing analysis of embryonic day (E) 14.5 cap stage molars revealed reductions in early expressed enamel matrix components (Odontogenic ameloblast-associated protein) and dentin dysplasia targets (Dentin matrix acidic phosphoprotein 1). "
9.	PhosphoproteinsIBA 01/01/2020 - "Whole-genome RNA-sequencing analysis of embryonic day (E) 14.5 cap stage molars revealed reductions in early expressed enamel matrix components (Odontogenic ameloblast-associated protein) and dentin dysplasia targets (Dentin matrix acidic phosphoprotein 1). "
10.	alpha 1 Chain Collagen Type IIBA 06/08/2021 - "Mutations in the gene encoding dentin sialophosphoprotein (DSPP) have been identified to cause dentinogenesis imperfecta (DGI) Types II and III and dentin dysplasia (DD) Type II. While DGI Type I is an OI-related syndromic phenotype caused mostly by monoallelic mutations in the genes encoding collagen type I alpha 1 chain (COL1A1) and collagen type I alpha 2 chain (COL1A2). "

Therapies and Procedures

1.	Therapeutics 08/28/2022 - "These findings suggest that in patients with XLH, oral management must take dentin dysplasia of the permanent teeth into consideration even if the patient's general condition is well controlled with conventional therapy." 11/17/2020 - "In patients with dentin dysplasia, conventional endodontic therapy is challenging. " 01/01/2019 - "The paper highlights anatomical and radiologicalaspects of dental abnormalities and emphasizes the significance of the educationof both general practitioners and paediatricians as regards referring patients withdiagnosed dentin dysplasia for a multi-specialty therapy."
2.	Crowns (Crown, Dental) 11/01/2014 - "Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by a defective dentin development with clinically normal-appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. " 01/07/2010 - "Dentin dysplasia is a rare hereditary disturbance of dentin formation characterized by defective dentin development with clinically normal appearing crowns, severe hypermobility of teeth and spontaneous dental abscesses or cysts. "
3.	Radiotherapy 05/01/1994 - "Other causes of rootless teeth such as odontodysplasia, dentin dysplasia, trauma, infection, radiation therapy, and neoplasia were excluded from her history and clinical and radiographic findings. "
4.	Mouth Rehabilitation 05/01/2014 - "A Case of Dentin Dysplasia with Full Mouth Rehabilitation: A 3-year Longitudinal Study. " 05/01/2014 - "Although literature reports suggest general guidelines for treatment planning, the present case report describes a full mouth rehabilitation of an 8-year-old female patient with dentin dysplasia. " 05/01/2014 - "A Case of Dentin Dysplasia with Full Mouth Rehabilitation: A 3-year Longitudinal Study."
5.	Bite Force (Occlusal Forces) 11/01/2021 - "Teeth affected by hereditary dentin dysplasia chip easily, even under normal masticatory forces; however, as a result of underlying sclerotic dentin formation and obliteration of pulp chambers in response to attrition, these teeth are not hypersensitive. "