MCID: DNT009
MIFTS: 35

Dentin Dysplasia malady

Genetic diseases, Oral diseases, Rare diseases categories

Aliases & Classifications for Dentin Dysplasia

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Aliases & Descriptions for Dentin Dysplasia:

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


Classifications:



External Ids:

Disease Ontology10 DOID:701
ICD9CM29 520.5
SNOMED-CT59 109492001, 45742009
MeSH36 D003805

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 dentin dysplasia, type i, with microdontia and misshapen teeth 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 Adhesion and Integrin cell surface interactions. Affiliated tissues include bone, and related mouse phenotypes are craniofacial and 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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Diseases in the Dentin Dysplasia family:

Dentin Dysplasia, Type Ii

Diseases related to Dentin Dysplasia via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 35)
idRelated DiseaseScoreTop Affiliating Genes
1dentin dysplasia, type i, with microdontia and misshapen teeth31.4DSPP, SMOC2
2dentin dysplasia, type ii30.7DMP1, DSPP, IBSP, SPP1
3dentinogenesis imperfecta29.3DMP1, DSPP, ENAM, IBSP, MSX2, SMOC2
4dentin dysplasia sclerotic bones10.5
5atypical dentin dysplasia due to smoc2 deficiency10.4
6amelogenesis imperfecta10.3
7dentinogenesis imperfecta type 310.1DMP1, DSPP
8solitary necrotic tumor of the liver10.1DSPP, ENAM
9rheumatoid arthritis10.1
10arthritis10.1
11dental fluorosis10.1
12hypervitaminosis d10.1
13calcinosis10.1
14periodontitis10.1
15familial tumoral calcinosis10.1
16pyogenic granuloma10.1
17taurodontism10.1
18tumoral calcinosis10.1
19hernia of ovary and fallopian tube10.1DSPP, ENAM
20methylmalonic aciduria and homocystinuria type cble10.1DSPP, SPP1
21intracranial liposarcoma10.1DSPP, SPP1
22pediatric intraocular retinoblastoma10.1DMP1, DSPP
23dental pulp disease10.1DSPP, ENAM
24dental enamel hypoplasia10.1DSPP, ENAM
25idiopathic panuveitis10.0DMP1, IBSP
26telangiectasis10.0DSPP, ENAM
27gastrointestinal tuberculosis10.0DSPP, ENAM
28movement disease9.9DSPP, ENAM
29childhood brain meningioma9.9DSPP, ENAM, SPP1
30pseudoxanthoma elasticum, forme fruste9.9IBSP, SPP1
31sapho syndrome9.8DSPP, IBSP, SPP1
32saethre-chotzen syndrome9.8IBSP, MSX2
33anal fistula9.8DSPP, ENAM, MSX2
34small cell cancer of the lung, somatic9.8IBSP, SPP1
35depressed scar9.7DMP1, DSPP, IBSP, SPP1

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



Diseases related to dentin dysplasia

Symptoms for Dentin Dysplasia

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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

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:00053828.1DMP1, DSPP, ENAM, MSX2
2MP:00053907.8DMP1, IBSP, MSX2, SPP1

Publications for Dentin Dysplasia

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Articles related to Dentin Dysplasia:

(show top 50)    (show all 64)
idTitleAuthorsYear
1
Dentin dysplasia type I - A rare entity. (26097326)
2015
2
Isolated dentinogenesis imperfecta and dentin dysplasia: revision of the classification. (25118030)
2014
3
Dentin dysplasia type 1d: a rare case. (25728124)
2014
4
Interradicular dentin dysplasia associated with amelogenesis imperfecta with taurodontism or trichodentoosseous syndrome: a diagnostic dilemma. (24992867)
2014
5
Rootless teeth: Dentin dysplasia type I. (24403801)
2013
6
Dentin dysplasia type I. (23814198)
2013
7
Inhabitual autosomal recessive form of dentin dysplasia type I in a large consanguineous Moroccan family. (23712319)
2013
8
Orthodontic treatment of a patient with dentin dysplasia type I. (23452977)
2013
9
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
10
Variation of dentin dysplasia type I: report of atypical findings in the permanent dentition. (21519653)
2011
11
Dentin dysplasia type I: a case report and review of the literature. (20205797)
2010
12
Dentin dysplasia: single-tooth involvement? (19417880)
2009
13
Dentin dysplasia type I with pyogenic granuloma in a 12-year-old girl. (19736509)
2009
14
Periodontal status of patients with dentin dysplasia type I: report of three cases within a family. (18597615)
2008
15
Overlapping DSPP mutations cause dentin dysplasia and dentinogenesis imperfecta. (19029076)
2008
16
Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. (19021896)
2008
17
Dentin dysplasia type I: a challenge for treatment with dental implants. (17714586)
2007
18
General practitioner's radiology case 55. Dentin dysplasia. (18019125)
2007
19
Phenotypic variation in dentinogenesis imperfecta/dentin dysplasia linked to 4q21. (16567553)
2006
20
Dentin dysplasia type I: report of atypical cases in the permanent and mixed dentitions. (15243476)
2004
21
Oral and maxillofacial pathology case of the month. Dentin dysplasia type I. (12592741)
2002
22
Dentin dysplasia, type II: report of 2 new families and review of the literature. (10397672)
1999
23
Case study. Dentin dysplasia. (10895705)
1999
24
Refined mapping of the human dentin sialophosphoprotein (DSPP) gene within the critical dentinogenesis imperfecta type II and dentin dysplasia type II loci. (9541230)
1998
25
Dentin dysplasia type I. Report of case and ultrastructural study. (9866969)
1998
26
Dentin dysplasia, type II linkage to chromosome 4q. (9493074)
1997
27
Dentin dysplasia--a case report. (9522734)
1995
28
Dentin dysplasia type I. Radiologic and genetic perspectives in a six-generation family. (7970601)
1994
29
Spectrum of dentin dysplasia in a family: case report and literature review. (7854952)
1994
30
Transmission electron microscopic appearance of dentin matrix in type II dentin dysplasia. (1754835)
1991
31
Dentin dysplasia: review of the literature and a proposed subclassification based on radiographic findings. (1891231)
1991
32
Type I dentin dysplasia: report of two cases. (1785908)
1991
33
Dentin dysplasia type II: absence of type III collagen in dentin. (2195160)
1990
34
Dentin dysplasia type II: report of case. (2760319)
1989
35
Clinical, radiographic, and histological manifestations of dentin dysplasia, type I: Report of case. (2592698)
1989
36
Amelogenesis imperfecta, dentinogenesis imperfecta and dentin dysplasia revisited: problems in classification. (3150442)
1988
37
A scanning electron microscopic study of dentin dysplasia type II in primary dentition. (6589579)
1984
38
Dentin dysplasia: endodontic considerations and report of involvement of three siblings. (6590748)
1984
39
Management of dentin dysplasia and facial disharmony. (6574606)
1983
40
Interrupted root development--a new form of dentin dysplasia. (6603951)
1983
41
Dentin dysplasia type I: a clinical report. (6947011)
1981
42
Dentin dysplasia type I: a scanning electron microscopic analysis of the primary dentition. (6935585)
1980
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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Pathways related to Dentin Dysplasia according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6DSPP, IBSP
29.5IBSP, SPP1
39.5IBSP, SPP1
48.8DMP1, DSPP, IBSP
5
Show member pathways
8.3DMP1, DSPP, IBSP, SPP1

GO Terms for genes affiliated with Dentin Dysplasia

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Cellular components related to Dentin Dysplasia according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1membrane-bounded vesicleGO:003198810.0IBSP, SPP1
2extracellular regionGO:00055768.3DMP1, DSPP, IBSP, SPP1
3proteinaceous extracellular matrixGO:00055788.2DMP1, DSPP, ENAM, SMOC2

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

idNameGO IDScoreTop Affiliating Genes
1cellular response to growth factor stimulusGO:00713639.5IBSP, MSX2
2osteoblast differentiationGO:00016499.2IBSP, MSX2, SPP1
3positive regulation of cell-substrate adhesionGO:00108119.1DMP1, SMOC2, SPP1
4biomineral tissue developmentGO:00312148.3DMP1, DSPP, ENAM, IBSP, SPP1
5extracellular matrix organizationGO:00301987.9DMP1, DSPP, IBSP, SMOC2, SPP1
6ossificationGO:00015037.8DMP1, DSPP, IBSP, MSX2, SPP1

Molecular functions related to Dentin Dysplasia according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix bindingGO:00508409.2DMP1, SPP1

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