Page last updated: 2024-09-27

Denys-Drash Syndrome

A disorder of sex development characterized by UROGENITAL ABNORMALITIES; GONADAL DYSGENESIS; and WILMS TUMOR. It is caused by a mutation in the Wilms tumor suppressor gene (GENES, WILMS TUMOR) on chromosome 11.

Synonyms(5)

Synonym
Wilms Tumor and Pseudohermaphroditism
Drash Syndrome
Nephropathy, Wilms Tumor, and Genital Anomalies
Denys-Drash Syndrome
Pseudohermaphroditism, Nephron Disorder and Wilms' Tumor

Research Excerpts

Research

Studies (18)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's8 (44.44)18.1394
2010's9 (50.00)28.8240
2020's1 (5.56)9.53
DrugIndicatedRelationship StrengthStudiesTrials
adenine0low20
cytosine0low10
midodrine0low10
glutamine0low10
histidine0low10
arginine0low10
methylprednisolone0low10
fluorescein-5-isothiocyanate0low10
biotin0low10
angiotensin ii0low10
tacrolimus0low10
dactinomycin0low10
transforming growth factor beta0low20
nephrin0low40
cyclosporine0low30
guanine0low20

Protein Targets (283)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE4004
Chain A, MTA/SAH nucleosidase0101
Chain A, Ribosome-inactivating protein alpha-trichosanthin0011
Chain A, Ricin A chain0011
Chain A, Ribosome-inactivating protein 30011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
Chain A, Phenylethanolamine N-methyltransferase0011
RAR-related orphan receptor gamma4004
TDP1 protein3003
AR protein6006
nuclear receptor subfamily 1, group I, member 32002
cytochrome P450 family 3 subfamily A polypeptide 42002
glucocorticoid receptor [Homo sapiens]4004
estrogen-related nuclear receptor alpha4004
pregnane X nuclear receptor3003
estrogen nuclear receptor alpha6006
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a4004
nuclear factor erythroid 2-related factor 2 isoform 22002
heat shock protein beta-12002
importin subunit beta-1 isoform 11001
snurportin-11001
tumor susceptibility gene 101 protein1001
GTP-binding nuclear protein Ran isoform 11001
lamin isoform A-delta101001
Voltage-dependent calcium channel gamma-2 subunit2002
Bile salt export pump0303
Cyclin-dependent kinase 10101
Heat shock protein HSP 90-beta0101
Glutamate receptor 22002
Xanthine dehydrogenase/oxidase0101
TAR DNA-binding protein 431001
Protein mono-ADP-ribosyltransferase PARP150101
Leucine-rich repeat serine/threonine-protein kinase 20202
ATPase family AAA domain-containing protein 54004
Ataxin-24004
Chain A, TYROSYL-DNA PHOSPHODIESTERASE1001
Carbonic anhydrase 10004
Carbonic anhydrase 20004
Alanine racemase, biosynthetic0001
5-hydroxytryptamine receptor 1D0002
Glutamate receptor ionotropic, NMDA 1 0315
Proton-coupled amino acid transporter 10202
Adenosine deaminase0001
Carbonic anhydrase 40002
glucocerebrosidase1001
DNA polymerase kappa isoform 12002
histone acetyltransferase KAT2A isoform 12002
Atrial natriuretic peptide receptor 30101
Type-1A angiotensin II receptor 0213
Type-1 angiotensin II receptor0011
Type-1B angiotensin II receptor0202
Type-1 angiotensin II receptor0224
Type-2 angiotensin II receptor0202
Type-2 angiotensin II receptor0202
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0022
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0022
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0022
thioredoxin reductase2002
phosphopantetheinyl transferase1001
NFKB1 protein, partial1001
GLS protein2002
thyroid stimulating hormone receptor1001
alpha-galactosidase1001
euchromatic histone-lysine N-methyltransferase 24004
cytochrome P450 2C19 precursor1001
chromobox protein homolog 11001
flap endonuclease 13003
DNA polymerase iota isoform a (long)1001
muscarinic acetylcholine receptor M11001
lethal factor (plasmid)1001
Cytochrome P450 3A40102
Nitric oxide synthase, endothelial0002
Nitric oxide synthase, brain0001
Nitric oxide synthase, brain 0001
Nitric oxide synthase, inducible0001
Nitric oxide synthase, inducible0013
Cationic amino acid transporter 30101
ATP-dependent phosphofructokinase3003
Chain A, Avidin0011
Chain A, Protein (streptavidin)0011
Chain B, Protein (streptavidin)0011
Chain A, Streptavidin0011
Chain D, Streptavidin0011
Chain A, Streptavidin0011
Chain D, Streptavidin0011
Chain A, Streptavidin0011
Chain D, Streptavidin0011
Chain A, Streptavidin Complex With Biotin0011
Chain D, Circularly Permuted Core-streptavidin E51/a460011
Chain A, Core-streptavidin0011
Chain D, Core-streptavidin0011
Chain A, Core-streptavidin0011
Chain D, Core-streptavidin0011
Chain A, Core-streptavidin0011
Chain D, Core-streptavidin0011
Chain A, Core-streptavidin0011
Chain D, Core-streptavidin0011
Chain A, Avidin0011
Chain A, Streptavidin0011
Chain B, Streptavidin0011
Chain A, Streptavidin0011
Chain B, Streptavidin0011
Chain A, JmjC domain-containing histone demethylation protein 3A1001
apical membrane antigen 1, AMA11001
nonstructural protein 10101
green fluorescent protein, partial0101
vitamin D3 receptor isoform VDRA2002
insulin-degrading enzyme isoform 10011
Streptavidin0011
Receptor-type tyrosine-protein phosphatase beta0001
GLI family zinc finger 33003
IDH12002
geminin3003
Deoxycytidine kinase0001
Chain A, Putative fructose-1,6-bisphosphate aldolase1001
Chain A, HADH2 protein1001
Chain B, HADH2 protein1001
Chain A, ATP-DEPENDENT DNA HELICASE Q11001
Chain A, Ferritin light chain2002
Chain A, Cruzipain1001
interleukin 81001
15-lipoxygenase, partial2002
hypoxia-inducible factor 1 alpha subunit3003
USP1 protein, partial2002
Microtubule-associated protein tau1001
caspase 7, apoptosis-related cysteine protease1001
aldehyde dehydrogenase 1 family, member A11001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)2002
estrogen receptor 2 (ER beta)1001
progesterone receptor3003
retinoic acid nuclear receptor alpha variant 11001
retinoid X nuclear receptor alpha3003
farnesoid X nuclear receptor1001
peroxisome proliferator-activated receptor delta2002
peroxisome proliferator activated receptor gamma3003
vitamin D (1,25- dihydroxyvitamin D3) receptor2002
caspase-31001
RAD510101
aryl hydrocarbon receptor1001
activating transcription factor 62002
v-jun sarcoma virus 17 oncogene homolog (avian)1001
Histone H2A.x2002
Caspase-71001
Bloom syndrome protein isoform 12002
thyroid hormone receptor beta isoform a1001
caspase-31001
thyroid hormone receptor beta isoform 22002
huntingtin isoform 21001
histone-lysine N-methyltransferase 2A isoform 2 precursor2002
nuclear factor erythroid 2-related factor 2 isoform 12002
nuclear receptor ROR-gamma isoform 12002
peripheral myelin protein 222002
survival motor neuron protein isoform d1001
cytochrome P450 3A4 isoform 12002
caspase-1 isoform alpha precursor1001
Gamma-aminobutyric acid receptor subunit pi2002
Interferon beta2002
Cellular tumor antigen p533003
ATP-dependent translocase ABCB10001
Gamma-aminobutyric acid receptor subunit beta-12002
Gamma-aminobutyric acid receptor subunit delta2002
Gamma-aminobutyric acid receptor subunit gamma-22002
Gamma-aminobutyric acid receptor subunit alpha-52002
Gamma-aminobutyric acid receptor subunit alpha-32002
Gamma-aminobutyric acid receptor subunit gamma-12002
Gamma-aminobutyric acid receptor subunit alpha-22002
Gamma-aminobutyric acid receptor subunit alpha-42002
Gamma-aminobutyric acid receptor subunit gamma-32002
Gamma-aminobutyric acid receptor subunit alpha-62002
Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 10101
Nuclear receptor ROR-gamma1001
Caspase-71001
Spike glycoprotein2002
Gamma-aminobutyric acid receptor subunit alpha-12002
Growth factor receptor-bound protein 20101
Gamma-aminobutyric acid receptor subunit beta-32002
Gamma-aminobutyric acid receptor subunit beta-22002
Pantothenate synthetase0101
Discoidin domain-containing receptor 20101
Growth factor receptor-bound protein 2 0101
Reverse transcriptase/RNaseH 0101
GABA theta subunit2002
Peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase0011
Gamma-aminobutyric acid receptor subunit epsilon2002
thyrotropin-releasing hormone receptor1001
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a1001
Chain A, Glutamine Binding Protein0011
arylsulfatase A1001
peripheral myelin protein 22 isoform 11001
Neutral amino acid transporter A0101
Asc-type amino acid transporter 10101
Neutral amino acid transporter B(0)0101
Carbonic anhydrase-like protein, putative0002
Amino acid transporter0101
Glycine receptor subunit alpha-10011
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent glycine transporter 10101
Glutamate receptor ionotropic, NMDA 2A 0214
Glutamate receptor ionotropic, NMDA 2B0214
Glutamate receptor ionotropic, NMDA 2C0214
Large neutral amino acids transporter small subunit 10202
Glutamate receptor ionotropic, NMDA 2D0214
Glutamate receptor ionotropic, NMDA 3B0214
Serine racemase0101
Olfactory receptor 51E20011
Glutamate receptor ionotropic, NMDA 3A0214
Sodium- and chloride-dependent glycine transporter 20101
Purine nucleoside phosphorylase0202
Hypoxanthine-guanine phosphoribosyltransferase0001
Ricin0101
2-amino-4-hydroxy-6-hydroxymethyldihydropteridine pyrophosphokinase0011
Purine nucleoside phosphorylase 0012
Shiga toxin subunit A0101
Guanine deaminase0001
Chain A, HISTIDINE-BINDING PROTEIN0011
ATAD5 protein, partial1001
Carbonic anhydrase 120001
Plasma kallikrein0001
Carbonic anhydrase 30001
Histidine-binding periplasmic protein0011
Carbonic anhydrase 40001
Carbonic anhydrase 60001
Adenosine receptor A10001
Adenosine receptor A2a0001
Carbonic anhydrase 5A, mitochondrial0001
Carbonic anhydrase0001
Carbonic anhydrase 70001
Carbonic anhydrase0001
Glutaminyl-peptide cyclotransferase0101
Carbonic anhydrase 90001
Carbonic anhydrase 0001
Carbonic anhydrase0001
Carbonic anhydrase0001
Carbonic anhydrase 0001
Carbonic anhydrase 130001
Carbonic anhydrase 150001
Carbonic anhydrase 130001
Carbonic anhydrase 140001
Carbonic anhydrase 5B, mitochondrial0001
acetylcholinesterase1001
PPM1D protein1001
nonstructural protein 11001
G1001
bromodomain adjacent to zinc finger domain 2B1001
nuclear receptor subfamily 1, group I, member 21001
muscleblind-like protein 1 isoform 11001
ATP-binding cassette sub-family C member 30202
Multidrug resistance-associated protein 40202
Nuclear receptor subfamily 1 group I member 20022
HLA class I histocompatibility antigen, B alpha chain 1001
Glucocorticoid receptor0202
Glycine receptor subunit alpha-10202
Androgen receptor0202
Glycine receptor subunit beta0202
Glycine receptor subunit alpha-20202
Glycine receptor subunit alpha-30202
Inositol hexakisphosphate kinase 11001
Canalicular multispecific organic anion transporter 10202
Nuclear receptor subfamily 1 group I member 20011
cytochrome P450 2C9, partial1001
UDP-glucuronosyltransferase 2B70001
Cytochrome P450 3A50101
UDP-glucuronosyltransferase 1A40001
Serine/threonine-protein kinase mTOR0101
Peptidyl-prolyl cis-trans isomerase FKBP1A0213
Peptidyl-prolyl cis-trans isomerase FKBP1B0011
Serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform0101
Peptidyl-prolyl cis-trans isomerase FKBP50101
Splicing factor 3B subunit 30101
Broad substrate specificity ATP-binding cassette transporter ABCG20101
Solute carrier organic anion transporter family member 1B10101
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]