MCID: DNT009
MIFTS: 36

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 i, with microdontia and misshapen teeth, 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, breast and thyroid, 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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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 top 50)    (show all 72)
idRelated DiseaseScoreTop Affiliating Genes
1dentinogenesis imperfecta28.4DMP1, DSPP, ENAM, IBSP, MSX2, SMOC2
2dentin dysplasia, type i, with microdontia and misshapen teeth12.8
3dentin dysplasia, type ii12.7
4dentin dysplasia sclerotic bones12.5
5atypical dentin dysplasia due to smoc2 deficiency12.4
6denys-drash syndrome11.8
7diastrophic dysplasia11.4
8mohr-tranebjaerg syndrome11.4
9sacral agenesis with vertebral anomalies10.2DSPP, SMOC2
10spinocerebellar ataxia, autosomal recessive 1810.2DMP1, DSPP
11huriez syndrome10.1DMP1, DSPP
12solitary necrotic tumor of the liver10.1DSPP, ENAM
13hernia of ovary and fallopian tube10.1DSPP, ENAM
14prostate cancer10.1
15arthritis10.1
16hypertrophic cardiomyopathy10.1
17prostatitis10.1
18cardiomyopathy10.1
19papillary extrahepatic bile duct adenocarcinoma10.1DSPP, ENAM
20primary bacterial infectious disease10.0DMP1, DSPP
21urethral urothelial papilloma10.0DSPP, ENAM
22nasal cavity disease9.9DSPP, ENAM
23schizophrenia9.9
24rheumatoid arthritis9.9
25neuroblastoma9.9
26obesity9.9
27alcohol dependence9.9
28wolff-parkinson-white syndrome9.9
29factor x deficiency9.9
30anaplastic large cell lymphoma9.9
31bipolar disorder9.9
32leukemia9.9
33polycystic ovary syndrome9.9
34lymphoma9.9
35ileitis9.9
36ptosis9.9
37siderosis9.9
38blepharophimosis9.9
39short bowel syndrome9.9
40polydactyly9.9
41myxedema9.9
42nephrocalcinosis9.9
43quadriplegia9.9
44sjogren's syndrome9.9
45cholecystitis9.9
46acromegaly9.9
47pancreas disease9.9
48acalculous cholecystitis9.9
49bruxism9.9
50churg-strauss syndrome9.9

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, Breast, Thyroid, Monocytes, Endothelial, B cells, Ovary

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 64)
idTitleAuthorsYear
1
RADIOSENSITIVITY IN LYMPHOBLASTOID CELL LINES DERIVED FROM SHWACHMAN-DIAMOND SYNDROME PATIENTS. (25870433)
2015
2
The inflammation, vascular repair and injury responses to exercise in fit males with and without Type 1 diabetes: an observational study. (26044827)
2015
3
True Precocious Puberty Following Treatment of a Leydig Cell Tumor: Two Case Reports and Literature Review. (26579503)
2015
4
Metastatic Rhabdomyosarcoma: Still Room for Improvement. (26573072)
2015
5
IL-13 receptor I+2 signaling requires a scaffold protein, FAM120A, to activate the FAK and PI3K pathways in colon cancer metastasis. (25896327)
2015
6
Serum adhesion molecules as outcome predictors in adult severe sepsis patients requiring mechanical ventilation in the emergency department. (24998754)
2014
7
miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production. (24940638)
2014
8
Johnson-McMillin Microtia Syndrome: New Additional Family. (25374870)
2014
9
Northern British Columbian Aboriginal mothers: raising adolescents with fetal alcohol spectrum disorder. (22802305)
2013
10
Prognosis of the intrahepatic cholangiocarcinoma after resection: hepatitis B virus infection and adjuvant chemotherapy are favorable prognosis factors. (24139471)
2013
11
Redox-dependent control of FOXO/DAF-16 by transportin-1. (23333309)
2013
12
Prediction of invasive candidal infection in critically ill patients with severe acute pancreatitis. (23506945)
2013
13
Primary hyperparathyroidism and acute pancreatitis. (23332718)
2013
14
The autoimmune basis of narcolepsy. (23725858)
2013
15
A novel definition of extrathyroidal invasion for patients with papillary thyroid carcinoma for predicting prognosis. (22402972)
2012
16
Seroprevalence and risk factors for Leptospirosis in goats in UberlAcndia, Minas Gerais, Brazil. (21647773)
2012
17
Giant head neurofibroma. (22201112)
2012
18
Abdominal compartment syndrome: potentially lethal and easy to miss. (21905464)
2011
19
Serological markers of sand fly exposure to evaluate insecticidal nets against visceral leishmaniasis in India and Nepal: a cluster-randomized trial. (21931871)
2011
20
Expression and clinical significance of androgen receptor in triple negative breast cancer. (20507730)
2010
21
Anterior chest wall giant cell tumor. (20058142)
2010
22
The myoepithelial cell: its role in normal mammary glands and breast cancer. (20235044)
2010
23
Identification of TCP10L as primate-specific gene derived via segmental duplication and homodimerization of TCP10L through the leucine zipper motif. (17377852)
2008
24
Extravasation of sclerosant after injection of N-butyl-2-cyanoacrylate for a bleeding gastric Dieulafoy lesion. (18633906)
2008
25
The prevalence of non-classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency in Greek women with hirsutism and polycystic ovary syndrome. (18187875)
2008
26
Unphosphorylated STAT6 contributes to constitutive cyclooxygenase-2 expression in human non-small cell lung cancer. (17237818)
2007
27
Young patients in a young nation: scarlet fever in early nineteenth century rural New England. (17411002)
2007
28
A phase 2/3 multicenter randomized clinical trial of ABX-CBL versus ATG as secondary therapy for steroid-resistant acute graft-versus-host disease. (17110457)
2007
29
Membrane lipid rafts coordinate estrogen-dependent signaling in human platelets. (17208317)
2007
30
Glucocorticoid receptor and vascular endothelial growth factor in nephrotic syndrome. (16825140)
2006
31
Fibrogenesis in alcoholic chronic pancreatitis: the role of tissue necrosis, macrophages, myofibroblasts and cytokines. (16680157)
2006
32
Imatinib mesylate inhibits osteoclastogenesis and joint destruction in rats with collagen-induced arthritis (CIA). (16816921)
2006
33
A urokinase-type plasminogen activator-inhibiting cyclic peptide with an unusual P2 residue and an extended protease binding surface demonstrates new modalities for enzyme inhibition. (16141208)
2005
34
Clopidogrel does not increase bleeding and allogenic blood transfusion in coronary artery surgery. (15019671)
2004
35
Modulation of gene expression induced in human epidermis by environmental stress in vivo. (14675196)
2003
36
The epidermal growth factor receptor pathway mediates resistance to sequential administration of radiation and chemotherapy in primary human glioblastoma cells in a RAS-dependent manner. (12154034)
2002
37
Alarming atrioventricular block and mitral valve prolapse in the Kearns-Sayre syndrome. (12007693)
2002
38
Differential expression of human nucleoside transporters in normal and tumor tissue. (11162617)
2001
39
Treating asthma in the new millennium. (19667533)
2000
40
Characterization and regional distribution of nitric oxide synthase in the human brain during normal ageing. (10407101)
1999
41
A neuronal subpopulation in the mammalian enteric nervous system expresses TrkA and TrkC neurotrophin receptor-like proteins. (9669764)
1998
42
Smoothelin, a novel cytoskeletal protein specific for smooth muscle cells. (8707825)
1996
43
Germline APC mutation (Gln1317) in a cancer-prone family that does not result in familial adenomatous polyposis. (8834176)
1996
44
X-linked mental retardation with thin habitus, osteoporosis, and kyphoscoliosis: linkage to Xp21.3-p22.12. (8826448)
1996
45
Office gynecology for the primary care physician, part II: pelvic pain, vulvar disease, disorders of menstruation, premenstrual syndrome, and breast disease. (8614176)
1996
46
Molecular cloning of RhD cDNA derived from a gene present in RhD- positive, but not RhD-negative individuals. (8329718)
1993
47
The cDNA sequence of mouse LAMP-2. Evidence for two classes of lysosomal membrane glycoproteins. (2318880)
1990
48
Retinal infarcts and haemorrhages due to scurvy. (2217046)
1990
49
Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts. (3609608)
1987
50
Ischaemic cerebrovascular accident and current indications for reconstructive procedures in the aortic arch arteries. (3454836)
1987

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