MCID: DNT009
MIFTS: 40

Dentin Dysplasia malady

Categories: Genetic diseases, Oral diseases, Rare diseases

Aliases & Classifications for Dentin Dysplasia

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Sources:
32LifeMap Discovery®, 10Disease Ontology, 47Novoseek, 12DISEASES, 51Orphanet, 36MeSH, 65UMLS, 27ICD10, 59SNOMED-CT, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Dentin Dysplasia:

Name: Dentin Dysplasia 32 10 47 12 51 36 65
Dentinal Dysplasia 10
 
Dd 51

Characteristics:

Orphanet epidemiological data:

51
dentin dysplasia:
Inheritance: Autosomal dominant; Age of onset: All ages; Age of death: normal life expectancy

Classifications:



External Ids:

Disease Ontology10 DOID:701
ICD1027 K00.5
MeSH36 D003805
Orphanet51 1653
SNOMED-CT59 109492001, 45742009
ICD10 via Orphanet28 K00.5
MESH via Orphanet37 D003805
UMLS via Orphanet66 C0011430
UMLS65 C0011430

Summaries for Dentin Dysplasia

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Disease Ontology:10 A teeth hard tissue disease characterized by presence of normal enamel but atypical dentin with abnormal pulpal morphology.

MalaCards based summary: Dentin Dysplasia, also known as dentinal dysplasia, is related to dentinogenesis imperfecta and dentin dysplasia, type ii, and has symptoms including abnormality of dental morphology, abnormality of dentin and increased bone mineral density. An important gene associated with Dentin Dysplasia is DSPP (Dentin Sialophosphoprotein), and among its related pathways are Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers and ECM-receptor interaction. Affiliated tissues include bone, ovary and endothelial, and related mouse phenotype skeleton.

Wikipedia:68 Dentin dysplasia is a genetic disorder of teeth, commonly exhibiting an autosomal dominant inheritance.... more...

Related Diseases for Dentin Dysplasia

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Graphical network of the top 20 diseases related to Dentin Dysplasia:



Diseases related to dentin dysplasia

Symptoms for Dentin Dysplasia

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Symptoms:

 51
  • tooth shape anomaly
  • dentine anomaly
  • osteosclerosis/osteopetrosis/bone condensation
  • autosomal dominant inheritance
  • exostoses

HPO human phenotypes related to Dentin Dysplasia:

id Description Frequency HPO Source Accession
1 abnormality of dental morphology hallmark (90%) HP:0006482
2 abnormality of dentin hallmark (90%) HP:0010299
3 increased bone mineral density hallmark (90%) HP:0011001
4 exostoses typical (50%) HP:0100777

Drugs & Therapeutics for Dentin Dysplasia

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Dentin Dysplasia


Cochrane evidence based reviews: dentin dysplasia

Genetic Tests for Dentin Dysplasia

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Anatomical Context for Dentin Dysplasia

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MalaCards organs/tissues related to Dentin Dysplasia:

33
Bone, Ovary, Endothelial, Prostate, Thyroid, B cells, Monocytes

LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Dentin Dysplasia:
id TissueAnatomical CompartmentCell Relevance
1 ToothDentinOdontoblasts Affected by disease

Animal Models for Dentin Dysplasia or affiliated genes

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MGI Mouse Phenotypes related to Dentin Dysplasia:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053907.5DMP1, ENAM, IBSP, MSX2, SPP1

Publications for Dentin Dysplasia

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Articles related to Dentin Dysplasia:

(show top 50)    (show all 66)
idTitleAuthorsYear
1
Dentin Dysplasia in Notum Knockout Mice. (26926082)
2016
2
Dentin dysplasia type I - A rare entity. (26097326)
2015
3
Dentin dysplasia type I-novel findings in deciduous and permanent teeth. (26693824)
2015
4
Isolated dentinogenesis imperfecta and dentin dysplasia: revision of the classification. (25118030)
2014
5
Dentin dysplasia type 1d: a rare case. (25728124)
2014
6
Interradicular dentin dysplasia associated with amelogenesis imperfecta with taurodontism or trichodentoosseous syndrome: a diagnostic dilemma. (24992867)
2014
7
Rootless teeth: Dentin dysplasia type I. (24403801)
2013
8
Dentin dysplasia type I. (23814198)
2013
9
Inhabitual autosomal recessive form of dentin dysplasia type I in a large consanguineous Moroccan family. (23712319)
2013
10
Orthodontic treatment of a patient with dentin dysplasia type I. (23452977)
2013
11
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. (22392858)
2012
12
Variation of dentin dysplasia type I: report of atypical findings in the permanent dentition. (21519653)
2011
13
Dentin dysplasia type I: a case report and review of the literature. (20205797)
2010
14
Dentin dysplasia: single-tooth involvement? (19417880)
2009
15
Periodontal status of patients with dentin dysplasia type I: report of three cases within a family. (18597615)
2008
16
Overlapping DSPP mutations cause dentin dysplasia and dentinogenesis imperfecta. (19029076)
2008
17
Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. (19021896)
2008
18
Dentin dysplasia type I: a challenge for treatment with dental implants. (17714586)
2007
19
General practitioner's radiology case 55. Dentin dysplasia. (18019125)
2007
20
Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. (16567553)
2006
21
Dentin dysplasia type I: report of atypical cases in the permanent and mixed dentitions. (15243476)
2004
22
Oral and maxillofacial pathology case of the month. Dentin dysplasia type I. (12592741)
2002
23
Dentin dysplasia, type II: report of 2 new families and review of the literature. (10397672)
1999
24
Case study. Dentin dysplasia. (10895705)
1999
25
Refined mapping of the human dentin sialophosphoprotein (DSPP) gene within the critical dentinogenesis imperfecta type II and dentin dysplasia type II loci. (9541230)
1998
26
Dentin dysplasia type I. Report of case and ultrastructural study. (9866969)
1998
27
Dentin dysplasia, type II linkage to chromosome 4q. (9493074)
1997
28
Dentin dysplasia--a case report. (9522734)
1995
29
Dentin dysplasia type I. Radiologic and genetic perspectives in a six-generation family. (7970601)
1994
30
Spectrum of dentin dysplasia in a family: case report and literature review. (7854952)
1994
31
Transmission electron microscopic appearance of dentin matrix in type II dentin dysplasia. (1754835)
1991
32
Dentin dysplasia: review of the literature and a proposed subclassification based on radiographic findings. (1891231)
1991
33
Type I dentin dysplasia: report of two cases. (1785908)
1991
34
Dentin dysplasia type II: absence of type III collagen in dentin. (2195160)
1990
35
Dentin dysplasia type II: report of case. (2760319)
1989
36
Clinical, radiographic, and histological manifestations of dentin dysplasia, type I: Report of case. (2592698)
1989
37
Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. (3150442)
1988
38
A scanning electron microscopic study of dentin dysplasia type II in primary dentition. (6589579)
1984
39
Dentin dysplasia: endodontic considerations and report of involvement of three siblings. (6590748)
1984
40
Management of dentin dysplasia and facial disharmony. (6574606)
1983
41
Interrupted root development--a new form of dentin dysplasia. (6603951)
1983
42
Dentin dysplasia type I: a clinical report. (6947011)
1981
43
Dentin dysplasia II. (297771)
1979
44
Radiographic manifestations of an unusual combination Types I and Type II dentin dysplasia. (272613)
1978
45
Focal odontoblastic dysplasia: dentin dysplasia type III? (271928)
1977
46
Dentin dysplasia, type II: a rare autosomal dominant disorder. (269353)
1977
47
Dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case. (1063351)
1976
48
Dentin dysplasia, type II, or dentin dysplasia, coronal type. (4531620)
1974
49
Polarized light study of enamel and dentin dysplasia. (5240796)
1968
50
Dentine dysplasia associated with rheumatoid arthritis and hypervitaminosis D. (13224191)
1955

Variations for Dentin Dysplasia

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Expression for genes affiliated with Dentin Dysplasia

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Search GEO for disease gene expression data for Dentin Dysplasia.

Pathways for genes affiliated with Dentin Dysplasia

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GO Terms for genes affiliated with Dentin Dysplasia

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Biological processes related to Dentin Dysplasia according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1biomineral tissue developmentGO:00312149.6DMP1, SPP1
2ossificationGO:00015039.5DSPP, MSX2
3cellular response to growth factor stimulusGO:00713639.2IBSP, MSX2

Sources for Dentin Dysplasia

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet