Gene Disease Score gda Association Type Original DB Sentence supporting the association PMID PMID Year
Entrez Id: 6499
Gene Symbol: SKIV2L
SKIV2L
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.670 Biomarker CTD_human

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Copeptin is not useful as marker of malignant disease in the syndrome of inappropriate antidiuresis. 31794422

2020

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE The reduction/absence of the functional FMRP protein, coded by the X-linked Fmr1 gene in humans, is responsible for the syndrome. 31680833

2019

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE The reduction/absence of the functional FMRP protein, coded by the X-linked <i>Fmr1</i> gene in humans, is responsible for the syndrome. 30815010

2019

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Copeptin and its role in the diagnosis of diabetes insipidus and the syndrome of inappropriate antidiuresis. 31004513

2019

Entrez Id: 3291
Gene Symbol: HSD11B2
HSD11B2
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE Mutations in the HSD11B2 gene encoding 11-HSD2 cause the syndrome of apparent mineralocorticoid excess, a severe form of familial hypertension. 29843121

2018

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Patients undergoing transsphenoidal pituitary surgery (TSS) are at risk for several serious complications, including the syndrome of inappropriate antidiuretic hormone and subsequent hyponatremia. 29075986

2018

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Safety and Efficacy of Tolvaptan in Korean Patients with Hyponatremia Caused by the Syndrome of Inappropriate Antidiuretic Hormone. 29629516

2018

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE A Rare Presentation of the Syndrome of Inappropriate Antidiuretic Hormone in a 12-Year-Old Girl as the Initial Presentation of an Immature Ovarian Teratoma. 28818586

2018

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 AlteredExpression BEFREE We report an unusual case of a premature neonate with persistent hyponatremia, markedly elevated plasma arginine vasopressin level (32.7 pg/mL), and clinical findings consistent with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). 30364227

2018

Entrez Id: 3291
Gene Symbol: HSD11B2
HSD11B2
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Congenital deficiency of 11β-HSD2 causes a form of salt-sensitive hypertension known as the syndrome of apparent mineralocorticoid excess. 28938454

2017

Entrez Id: 7157
Gene Symbol: TP53
TP53
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE These findings suggest a comprehensive multi-organ screening program for people with TP53 mutations provides psychological benefit independent of an impact on cancer morbidity and mortality associated with the syndrome. 28124295

2017

Entrez Id: 7157
Gene Symbol: TP53
TP53
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE The frequency of LFS/LFL families with no TP53 mutations detected suggests the involvement of other genes in the syndrome. 27714481

2017

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Tolvaptan use in cancer patients with hyponatremia due to the syndrome of inappropriate antidiuretic hormone: a post hoc analysis of the SALT-1 and SALT-2 trials. 28251822

2017

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Overall, the study confirms the presence of abnormalities in vocalization behavior in adult Fmr1 KO mice that we believe are consistent with communication deficits seen in the syndrome. 28552599

2017

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 PosttranslationalModification BEFREE The most common etiology of the syndrome is expansion and methylation of a CGG trinucleotide at chromosome region Xq27.3 involving FMR1 (fragile X mental retardation 1 gene). 27667322

2016

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 AlteredExpression BEFREE In FXS, the fragile X mental retardation 1 (FMR1) gene is silenced and the fragile X mental retardation protein (FMRP) is not expressed, resulting in the characteristic features of the syndrome. 24232444

2014

Entrez Id: 7157
Gene Symbol: TP53
TP53
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE This review discusses LFS, describes its association with TP53, and examines the classic and evolving definitions of the syndrome. 24706533

2014

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE Here, we show that fragile X (Fmr1) knockout mice display abnormalities in the myelination of cerebellar axons as early as the first postnatal week, corresponding roughly to the equivalent time in human brain development when symptoms of the syndrome first become apparent (1-3 years of age). 23740941

2013

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE The syndrome is due to mutations of the FMR1 gene that result in the absence of fragile X mental retardation protein (FMRP). 23660422

2013

Entrez Id: 2332
Gene Symbol: FMR1
FMR1
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE The predominant cause of the syndrome is the loss of a single protein, FMRP (Fragile X mental retardation protein). 20298211

2010

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 Biomarker BEFREE In patients and 32 healthy control subjects, plasma copeptin concentration and standard biochemical parameters were tested for their utility of diagnosing the syndrome of inappropriate antidiuresis (SIAD). 18984663

2009

Entrez Id: 7157
Gene Symbol: TP53
TP53
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE Germline TP53 mutations are found in only 70% of families with the Li-Fraumeni syndrome (LFS), and with an even lower frequency in families suggestive of LFS but not meeting clinical criteria of the syndrome. 19602465

2009

Entrez Id: 551
Gene Symbol: AVP
AVP
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 AlteredExpression BEFREE Patients with these novel gain-of-function V2R mutations have a disorder which we have termed "nephrogenic syndrome of inappropriate antidiuresis" (NSIAD): a clinical presentation consistent with the syndrome of inappropriate antidiuretic hormone secretion but with undetectable levels of arginine vasopressin (antidiuretic hormone). 17261972

2006

Entrez Id: 3291
Gene Symbol: HSD11B2
HSD11B2
CUI: C1857276
Disease: Trichohepatoenteric Syndrome
Trichohepatoenteric Syndrome
0.100 GeneticVariation BEFREE Mutations in the HSD11B2 gene cause the syndrome of apparent mineralocorticoid excess, an autosomal recessive form of inherited hypertension in which cortisol acts as a potent mineralocorticoid. 17035606

2006