Dosage-Sensitive Sex Reversal (DSS) malady

Summaries for Dosage-Sensitive Sex Reversal

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MalaCards: Dosage-Sensitive Sex Reversal, also known as DSS, is related to dengue shock syndrome and leydig cells hypoplasia. An important gene associated with Dosage-Sensitive Sex Reversal is NR0B1 (nuclear receptor subfamily 0, group B, member 1), and among its related pathways are Pregnenolone biosynthesis and PKA activation in glucagon signalling. The compounds estrogen and cycloheximide have been mentioned in the context of this disorder. Related mouse phenotypes are endocrine/exocrine gland and embryogenesis.

Aliases & Classifications for Dosage-Sensitive Sex Reversal

43NIH Rare Diseases, 61UMLS
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Aliases & Descriptions:

dosage-sensitive sex reversal 43 61
dss 43

Related Diseases for Dosage-Sensitive Sex Reversal

17GeneCards, 18GeneDecks
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Diseases related to Dosage-Sensitive Sex Reversal via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 62)
idRelated DiseaseScoreTop Affiliating Genes
1dengue shock syndrome10.4
2leydig cells hypoplasia10.3
3nr0b1-related 46,xy dsd and 46,xy cgd10.3
4dengue hemorrhagic fever10.3
5hemorrhagic fever10.3
6charcot-marie-tooth disease type 4e10.2
7ulcerative colitis10.1
8lipoid adrenal hyperplasia10.0STAR
10hypogonadism10.0NR0B1, NR5A1
11gonadal dysgenesis10.0NR0B1, NR5A1
13congenital adrenal insufficiency10.0STAR, CYP11A1
14kallmann syndrome10.0ESRRB, NR0B1, NR5A1
15cholestasis10.0NR1H2, NR0B2
1646,xy disorder of sex development and 46,xy complete gonadal dysgenesis10.0WNT4, NR0B1, NR5A1
17prolactinoma10.0ESR1, NR5A1
18ovarian disease10.0STAR, ESR1
19congenital adrenal hyperplasia10.0STAR, NR5A1, CYP11A1
21pituitary tumors10.0ESR1, NR1H2
22hyperandrogenism10.0CYP11A1, NR3C1
23wilms tumor10.0CTNNB1, NR5A1, NR0B1
24x-linked adrenal hypoplasia congenita10.0STAR, NR5A1, NR0B1, ESRRB
25premature ovarian failure10.0CYP11A1, STAR
26choriocarcinoma10.0STAR, NR5A1, CYP11A1
27leydig cell tumor10.0STAR, NR5A1, NR0B1, CYP11A1
28embryonal cancer10.0NR5A1, NR1H2, ESRRB
29adrenocortical carcinoma10.0CYP11A1, NR0B1, NR5A1, STAR
30pituitary adenoma10.0NR0B1, NR3C1, NR5A1
31leukemia10.0NR3C1, NR1H2
32osteoarthritis10.0NR3C1, NR1H2
33melanoma10.0NR1H2, CTNNB1
34adenoma10.0CYP11A1, NR0B1, NR5A1, CTNNB1
35polycystic ovary syndrome10.0CYP11A1, NR1H2, NR5A1, STAR
36discrete subaortic stenosis10.0
37charcot-marie-tooth disease type 410.0
38adult syndrome10.0
39n syndrome10.0
40charcot-marie-tooth disease type 1b10.0
41storm syndrome10.0
42dengue fever, protection against10.0
43charcot-marie-tooth disease, type 4f10.0
44cushing's syndrome10.0NR3C1, NR0B1, CYP11A1
45endometriosis10.0ESR1, CYP11A1, NR5A1, STAR
46abrikosov's tumor10.0STAR, NR5A1, CYP11A1, ESR1
47retinoblastoma10.0ESR1, TRRAP, NR1H2
48osteosarcoma10.0ESR1, NR3C1
49insulin resistance10.0NR3C1, NR1H2, NR0B2
50cervical cancer10.0NR1H2, NR3C1

Graphical network of the top 20 diseases related to Dosage-Sensitive Sex Reversal:

Diseases related to dosage-sensitive sex reversal

Clinical Features for Dosage-Sensitive Sex Reversal

Drugs & Therapeutics for Dosage-Sensitive Sex Reversal

5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Search NIH Clinical Center for Dosage-Sensitive Sex Reversal

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Genetic Tests for Dosage-Sensitive Sex Reversal

Anatomical Context for Dosage-Sensitive Sex Reversal

Animal Models for Dosage-Sensitive Sex Reversal or affiliated genes

37MGI, 28inGenious Targeting Laboratory
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Publications for Dosage-Sensitive Sex Reversal

Genetic Variations for Dosage-Sensitive Sex Reversal

Expression for genes affiliated with Dosage-Sensitive Sex Reversal

1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dosage-Sensitive Sex Reversal

Search GEO for disease gene expression data for Dosage-Sensitive Sex Reversal.

Pathways for genes affiliated with Dosage-Sensitive Sex Reversal

54Reactome, 12EMD Millipore, 38NCBI BioSystems Database, 30KEGG
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Compounds for genes affiliated with Dosage-Sensitive Sex Reversal

45Novoseek, 60Tocris Bioscience, 29IUPHAR, 11DrugBank, 24HMDB, 50PharmGKB
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Compounds related to Dosage-Sensitive Sex Reversal according to GeneCards/GeneDecks:

(show top 50)    (show all 75)
idCompoundScoreTop Affiliating Genes
2cycloheximide4510.3STAR, NR5A1
3di-n-butyl phthalate4510.2CYP11A1, STAR
4monobutyl phthalate4510.2CYP11A1, STAR
5ecdysone4510.0NR5A1, NR1H2, ESRRB
6phtpp60 2910.9ESR1, ESRRB
727-hydroxycholesterol45 2910.9NR1H2, STAR, CYP11A1
8dpn609.9ESRRB, ESR1
9ppt60 2910.9ESR1, ESRRB
10ici 182,780609.9ESR1, ESRRB
11(z)-4-hydroxytamoxifen609.8ESR1, ESRRB
123beta-hydroxysteroid459.8STAR, ESRRB, NR5A1, CYP11A1
1322r-hydroxycholesterol45 2910.7STAR, CYP11A1, NR1H2, NR5A1
14ouabain45 1110.7CTNNB1, STAR, CYP11A1
15androstane459.7NR3C1, NR0B2, NR1H2
16chenodeoxycholic acid45 29 11 2412.6NR0B2, ESRRB, NR1H2
17cyproteroneacetate459.6CTNNB1, NR3C1, NR1H2
18aminoglutethimide45 1110.6NR3C1, CYP11A1, ESR1
19dhea459.5NR3C1, NR5A1, CYP11A1, STAR
20diethylstilbestrol45 29 1111.5STAR, NR5A1, ESR1, ESRRB
21rifampicin45 29 6011.5ESRRB, NR1H2, NR0B2, NR3C1
2217beta-hydroxysteroid459.5NR5A1, ESR1, STAR, CYP11A1
23bicalutamide45 60 1111.5NR1H2, ESR1, CTNNB1
24ketoconazole45 29 11 2412.5NR1H2, NR3C1, CYP11A1
25sterol459.4NR1H2, ESRRB, CYP11A1, NR5A1, STAR
26phenobarbital45 29 1111.4CTNNB1, NR1H2, NR3C1, ESRRB
27steroidal459.3NR3C1, NR1H2, ESR1, ESRRB
28leptomycin b45 6010.3NR3C1, NR1H2, CTNNB1
29corticosterone45 60 2411.3NR3C1, STAR, NR5A1, NR1H2, CYP11A1
305-aza-2deoxycytidine459.2NR5A1, NR1H2, ESR1, CTNNB1
31fluoxymesterone45 1110.2NR3C1, ESR1
32acth459.1NR5A1, NR3C1, ESRRB, STAR, CYP11A1, NR0B1
33geldanamycin45 50 60 1112.1NR1H2, NR3C1, ESR1, CTNNB1
34mifepristone45 60 29 1112.1CTNNB1, NR3C1, ESR1, NR1H2
35progestins459.1NR1H2, ESR1, NR3C1
36progestin459.0NR1H2, NR3C1, ESR1, CYP11A1, STAR
37actinomycin d458.9NR5A1, CYP11A1, ESR1, NR3C1, STAR
38cholesterol45 29 11 2411.9NR1H2, STAR, NR0B2, ESRRB, CYP11A1, NR0B1
394-hydroxytamoxifen458.9NR1H2, CTNNB1, ESR1, ESRRB, NR3C1
40dihydrotestosterone45 29 11 2411.9CYP11A1, NR3C1, ESR1, NR1H2, CTNNB1
41mg 13245 609.8STAR, CTNNB1, NR1H2, NR3C1, ESR1
42vitamin d458.8NR3C1, NR5A1, ESR1, CTNNB1, NR1H2
43dexamethasone45 50 29 1111.8CYP11A1, NR0B1, STAR, NR3C1, NR1H2, CTNNB1
44steroid458.7NR5A1, ESRRB, ESR1, STAR, NR0B1, NR0B2
45retinoid458.7ESRRB, NR3C1, NR1H2, CTNNB1, NR5A1, ESR1
46estradiol45 11 2410.5NR1H2, STAR, NR5A1, NR3C1, CYP11A1, ESR1
47gnrh458.3CTNNB1, STAR, NR5A1, NR1H2, NR0B1, CYP11A1
48retinoic acid45 249.3ESRRB, CYP11A1, NR3C1, NR1H2, CTNNB1, ESR1
49testosterone45 60 11 2410.8NR5A1, CTNNB1, STAR, NR1H2, NR3C1, NR0B1
50progesterone45 60 29 11 2411.6NR5A1, CTNNB1, STAR, NR1H2, NR3C1, NR0B1

GO Terms for genes affiliated with Dosage-Sensitive Sex Reversal

16Gene Ontology
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Cellular components related to Dosage-Sensitive Sex Reversal according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1mitochondrial cristaGO:0300619.8CYP11A1, STAR
2transcription factor TFTC complexGO:0332769.6TRRAP, KAT2A
3STAGA complexGO:0309149.3TRRAP, KAT2A
4nucleoplasmGO:0056547.8ESRRB, CTNNB1, NR0B2, NR5A1, NR1H2, NR3C1

Biological processes related to Dosage-Sensitive Sex Reversal according to GeneCards/GeneDecks:

(show all 33)
idNameGO IDScoreTop Affiliating Genes
1fractalkine metabolic processGO:05075610.3CYP11A1, STAR
2response to fungicideGO:06099210.2STAR, CYP11A1
3dibenzo-p-dioxin metabolic processGO:01889410.2STAR, CYP11A1
4biphenyl metabolic processGO:01887910.2STAR, CYP11A1
5regulation of steroid biosynthetic processGO:05081010.2STAR, NR5A1
6testosterone biosynthetic processGO:06137010.2STAR, CYP11A1
7phthalate metabolic processGO:01896310.2STAR, CYP11A1
8phenol-containing compound metabolic processGO:01895810.2STAR, CYP11A1
9progesterone biosynthetic processGO:00670110.2STAR, CYP11A1
10C21-steroid hormone biosynthetic processGO:00670010.2STAR, CYP11A1
11renal vesicle formationGO:07203310.1WNT4, CTNNB1
12cellular response to follicle-stimulating hormone stimulusGO:07137210.1CYP11A1, STAR
13Leydig cell differentiationGO:03332710.1NR0B1, CYP11A1
14intracellular receptor signaling pathwayGO:03052210.1NR5A1, NR0B1, ESRRB
15cellular response to antibioticGO:07123610.1STAR, CYP11A1
16estrogen biosynthetic processGO:00670310.1STAR, CYP11A1
17glucocorticoid metabolic processGO:00821110.0STAR, NR3C1
18oocyte developmentGO:04859910.0WNT4, CTNNB1
19cellular response to cadmium ionGO:07127610.0CYP11A1, STAR
20cellular response to transforming growth factor beta stimulusGO:0715609.9STAR, CYP11A1, WNT4
21response to corticosterone stimulusGO:0514129.9STAR, CYP11A1
22steroid biosynthetic processGO:0066949.9NR0B1, STAR
23cholesterol metabolic processGO:0082039.9CYP11A1, NR0B2, STAR
24negative regulation of sequence-specific DNA binding transcription factor activityGO:0434339.8NR0B2, NR0B1, ESR1
25cellular response to fibroblast growth factor stimulusGO:0443449.8CYP11A1, STAR
26smooth muscle cell differentiationGO:0511459.8CTNNB1, WNT4
27histone deubiquitinationGO:0165789.7KAT2A, TRRAP
28adrenal gland developmentGO:0303259.6NR5A1, NR3C1, NR0B1, WNT4
29male gonad developmentGO:0085849.3STAR, NR5A1, NR0B1, WNT4, ESR1
30positive regulation of transcription from RNA polymerase II promoterGO:0459448.9CTNNB1, NR5A1, NR1H2, ESR1, ESRRB
31negative regulation of transcription, DNA-dependentGO:0458928.9CTNNB1, NR1H2, NR0B1, WNT4
32transcription initiation from RNA polymerase II promoterGO:0063678.6ESRRB, ESR1, NR0B1, NR3C1, NR1H2, NR5A1
33gene expressionGO:0104678.6NR0B2, NR5A1, NR1H2, NR3C1, NR0B1, ESR1

Molecular functions related to Dosage-Sensitive Sex Reversal according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1retinoid X receptor bindingGO:0469659.9NR1H2, NR0B2
2ligand-activated sequence-specific DNA binding RNA polymerase II transcription factor activityGO:0048799.6NR5A1, NR1H2, NR0B1, ESRRB
3steroid bindingGO:0054969.3NR3C1, ESR1, ESRRB
4transcription factor bindingGO:0081349.0CTNNB1, NR0B1, ESR1, ESRRB
5sequence-specific DNA bindingGO:0435659.0ESRRB, NR0B1, NR3C1, NR1H2, NR5A1
6steroid hormone receptor activityGO:0037078.8ESRRB, ESR1, NR0B1, NR1H2, NR5A1, NR0B2
7transcription coactivator activityGO:0037138.8ESRRB, TRRAP, NR5A1, CTNNB1, KAT2A
8chromatin bindingGO:0036828.5ESR1, NR5A1, CTNNB1, KAT2A

Products for genes affiliated with Dosage-Sensitive Sex Reversal

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Sources for Dosage-Sensitive Sex Reversal

26ICD10 via Orphanet
36MESH via Orphanet
48OMIM via Orphanet
58SNOMED-CT via Orphanet
62UMLS via Orphanet