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Dentinogenesis Imperfecta

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An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.

Also Known As:

Dentin, Hereditary Opalescent; Dentinogenesis Imperfectas; Dentins, Hereditary Opalescent; Hereditary Opalescent Dentins; Imperfecta, Dentinogenesis; Imperfectas, Dentinogenesis; Opalescent Dentin, Hereditary; Opalescent Dentins, Hereditary; Hereditary Opalescent Dentin

Networked: 85 relevant articles (0 outcomes, 13 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Osteogenesis Imperfecta (Lobstein Disease)
2.	Anodontia
3.	Metabolic Bone Diseases (Osteopenia)
4.	Dentin Dysplasia
5.	Amelogenesis Imperfecta

Experts

1.	Simmer, James P: 5 articles (01/2012 - 05/2007)
2.	Hu, Jan C-C: 4 articles (01/2012 - 05/2007)
3.	MacDougall, Mary: 3 articles (01/2005 - 07/2003)
4.	Wang, Shih-Kai: 2 articles (01/2012 - 12/2011)
5.	Chan, Hui-Chen: 2 articles (01/2012 - 12/2011)
6.	Yamakoshi, Yasuo: 2 articles (01/2011 - 01/2011)
7.	Yamakoshi, Fumiko: 2 articles (01/2011 - 01/2011)
8.	Xie, Yixia: 2 articles (10/2010 - 04/2004)
9.	Lu, Yongbo: 2 articles (10/2010 - 04/2004)
10.	Feng, Jian Q: 2 articles (10/2010 - 04/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Dentinogenesis Imperfecta:

1.	CollagenIBA 01/01/1998 - "The aim of our study was to find the prevalence of dentinogenesis imperfecta (DI) and other dental anomalies in 88 patients with OI, to compare clinical with radiologic abnormalities, and to correlate these clinical/radiologic findings with the results of gel electrophoresis and molecular studies of collagen I. Twenty-eight percent of OI patients had DI. " 01/01/2014 - "Dentinogenesis imperfecta determines structural alterations of the collagen structure still not completely elucidated. " 12/01/1994 - "Altered collagen expression in human dentin: increased reactivity of type III and presence of type VI in dentinogenesis imperfecta, as revealed by immunoelectron microscopy." 07/01/1980 - "These results indicate that either dentin collagen is altered, or noncollagenous matrices associated with collagen are increased in dentinogenesis imperfecta dentin." 08/01/2005 - "The mouse mutation fragilitas ossium (fro) leads to a syndrome of severe osteogenesis and dentinogenesis imperfecta with no detectable collagen defect. "
2.	dentin sialophosphoproteinIBA 01/01/2010 - "Several studies have shown that the clinical phenotypes of dentinogenesis imperfecta type II (DGI-II) may be caused by mutations in dentin sialophosphoprotein (DSPP). " 01/01/2009 - "The aim of this study was to perform phenotype analysis and dentin sialophosphoprotein (DSPP) mutational analysis on 3 Brazilian families diagnosed with dentinogenesis imperfecta type II (DGI-II) attending the Dental Anomalies Clinic in Brasilia, Brazil. " 04/01/2011 - "Novel dentin phosphoprotein frameshift mutations in dentinogenesis imperfecta type II." 03/09/2009 - "A novel splice site mutation in the dentin sialophosphoprotein gene in a Chinese family with dentinogenesis imperfecta type II." 10/01/2006 - "Splicing site mutations in dentin sialophosphoprotein causing dentinogenesis imperfecta type II."
3.	Amino Acids FDA Link 10/01/2010 - "Unexpectedly, the deletion of nucleotides 1484 to 1490 (c.1484_1490delCTATCAC, delMut, resulting in replacement of the last 18 residues with 33 random amino acids) showed a severe dentin and enamel defect similar to a dentinogenesis imperfecta (DI) III-like phenotype. " 06/01/2010 - "Patients with mutations affecting the first 120 amino acids at the amino-terminal end of the collagen type I triple helix had blue sclera but did not have dentinogenesis imperfecta. " 05/15/1997 - "The dentin matrix acidic phosphoprotein 1 (DMP1) gene has been mapped to human chromosome 4q21 and shown to exhibit no recombination with the autosomal dominant disorder of dentin formation, dentinogenesis imperfecta type II. In the current study, sequencing of DMP1 cDNA and genomic clones has indicated that the human gene contains an open reading frame of 1539 bp, which predicts a highly acidic, serine-rich protein of 513 amino acids. "
4.	Opalescent dentinIBA 06/01/2008 - "Dentinogenesis imperfecta is an autosomal dominant disorder of tooth development characterized by the presence of opalescent dentin, resulting in a dusky blue to brownish discoloration of the teeth. " 06/01/1985 - "Evidence from this family supports the hypothesis that Brandywine isolate hereditary opalescent dentin is a variant of opalescent dentin." 06/01/1985 - "Confirmation of obliterated pulp chambers in the deciduous teeth of the mother of a child with Brandywine isolate hereditary opalescent dentin makes it unlikely that classic opalescent dentin and Brandywine isolate hereditary opalescent dentin are separate genetic disorders. " 06/01/1985 - "The mothers of both children and several other family members have classic opalescent dentin (dentinogenesis imperfecta type II). " 06/01/1985 - "An unusual presentation of opalescent dentin and Brandywine isolate hereditary opalescent dentin in an Ashkenazic Jewish family."
5.	Nonsense Codon (Nonsense Mutation)IBA 09/01/2006 - "The objective of this study was to characterize dentin ultrastructural features resulting from a nonsense mutation in DSPP gene and to define various phenotypes associated with specific DSPP mutations in families with Dentinogenesis Imperfecta type II (DGI-II). " 02/01/2001 - "We identified a nonsense mutation (Gln45stop) in exon 3 of the dentin sialophosphoprotein (DSPP) gene in a Chinese family with dentinogenesis imperfecta Shields type II (DGI-II), in which the affected members showed discoloration and severe attrition of their teeth, with obliterated pulp chambers."
6.	Protein Sorting Signals (Signal Peptide)IBA 10/01/2002 - "As recent studies have shown that dentinogenesis imperfecta type II results from mutation of the bicistronic dentine sialophosphoprotein gene (DSPP ), we have tested this hypothesis by sequencing DSPP in a family with a history of dentine dysplasia type II. Our results have shown that a missense change, which causes the substitution of a tyrosine for an aspartic acid in the hydrophobic signal peptide domain of the protein, underlies the phenotype in this family. "
7.	Calcitriol Receptors (Calcitriol Receptor)IBA 09/01/2013 - "The aim of this study is to manifest briefly DGI-II and to investigate the association between BsmI, TaqI and FokI polymorphisms of Vitamin D receptor (VDR) gene and dentinogenesis imperfecta type II in a Turkish family by PCR-RFLP methodology. " 09/01/2013 - "Evaluation of vitamin D receptor (VDR) gene polymorphisms (FokI, TaqI and ApaI) in a family with dentinogenesis imperfecta."
8.	DiamondIBA 03/01/2006 - "Four third molars extracted from two 19 year-old subjects (one patient affected by Dentinogenesis Imperfecta type II) were included in resin, two divided in slices with a air-cooled diamond disc, and two used to study the characterisation of the resin-dentin interface. "
9.	Complementary DNA (cDNA)IBA 05/15/1997 - "The dentin matrix acidic phosphoprotein 1 (DMP1) gene has been mapped to human chromosome 4q21 and shown to exhibit no recombination with the autosomal dominant disorder of dentin formation, dentinogenesis imperfecta type II. In the current study, sequencing of DMP1 cDNA and genomic clones has indicated that the human gene contains an open reading frame of 1539 bp, which predicts a highly acidic, serine-rich protein of 513 amino acids. "
10.	Tyrosine (L-Tyrosine)FDA Link 10/01/2002 - "As recent studies have shown that dentinogenesis imperfecta type II results from mutation of the bicistronic dentine sialophosphoprotein gene (DSPP ), we have tested this hypothesis by sequencing DSPP in a family with a history of dentine dysplasia type II. Our results have shown that a missense change, which causes the substitution of a tyrosine for an aspartic acid in the hydrophobic signal peptide domain of the protein, underlies the phenotype in this family. "

Therapies and Procedures

1.	Prosthodontics 04/01/2011 - "Dentinogenesis imperfecta and its prosthodontic management is a challenging task. " 04/01/2011 - "Prosthodontic rehabilitation of dentinogenesis imperfecta." 08/01/2009 - "[Pedigree analysis and prosthodontic treatment of patients with dentinogenesis imperfecta type II]." 08/01/2009 - "To study the heritable character and explore the prosthodontic treatment of opalescent dentin, also known as dentinogenesis imperfecta type II(DGI-II). "
2.	Overlay Denture 01/01/1998 - "Oral rehabilitation in dentinogenesis imperfecta with overdentures: case report." 12/01/1992 - "Most previous reports on dentinogenesis imperfecta describe treatment with overdentures, which have several disadvantages. " 04/01/1988 - "The use of overdentures in patients with dentinogenesis imperfecta." 01/01/1978 - "Removable overdentures in the oral rehabilitation of patients with dentinogenesis imperfecta." 04/01/2011 - "Although various reports in the literature suggest general guidelines for treatment planning, the present case report describes a full mouth rehabilitation of a young patient with dentinogenesis imperfecta treated by maxillary fixed partial dentures and mandibular fiber reinforced overdenture with metal occlusal surfaces."
3.	Mouth Rehabilitation 10/01/2014 - "Full-mouth rehabilitation for a patient with dentinogenesis imperfecta: a clinical report." 10/01/2002 - "All-ceramic restorations for complete-mouth rehabilitation in dentinogenesis imperfecta: a case report." 09/01/2012 - "Dentinogenesis imperfecta: full-mouth rehabilitation using implants and sinus grafts--a case report." 04/01/2011 - "Although various reports in the literature suggest general guidelines for treatment planning, the present case report describes a full mouth rehabilitation of a young patient with dentinogenesis imperfecta treated by maxillary fixed partial dentures and mandibular fiber reinforced overdenture with metal occlusal surfaces."
4.	Transplants (Transplant) 09/01/2012 - "Dentinogenesis imperfecta: full-mouth rehabilitation using implants and sinus grafts--a case report."
5.	Synchrotrons 06/01/2001 - "High-resolution synchrotron radiation computed tomography (SRCT) and small-angle x-ray scattering (SAXS) were performed on normal and dentinogenesis imperfecta type II (DI-II) teeth. "