|
CHOLESTASIS, PROGRESSIVE FAMILIAL INTRAHEPATIC, 5
|
0.700 |
GermlineCausalMutation
|
disease |
ORPHANET |
Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis.
|
26888176 |
2016 |
|
CHOLESTASIS, PROGRESSIVE FAMILIAL INTRAHEPATIC, 5
|
0.700 |
Biomarker
|
disease |
CTD_human |
|
|
|
|
CHOLESTASIS, PROGRESSIVE FAMILIAL INTRAHEPATIC, 5
|
0.700 |
CausalMutation
|
disease |
CLINVAR |
|
|
|
|
CHOLESTASIS, PROGRESSIVE FAMILIAL INTRAHEPATIC, 5
|
0.700 |
Biomarker
|
disease |
GENOMICS_ENGLAND |
|
|
|
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
The regulation about MDR3 mediated by FXR or PPARα in cholestasis is clear, but the mechanism through miRNA is hardly reported.
|
30964181 |
2019 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Targeting FXR with small molecules has been exploited to treat lipid-related disorders and diseases such as cholestasis, gallstones and hepatic disorders.
|
30393515 |
2018 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Despite the significant number of patent applications claiming steroidal and non-steroidal FXR agonists, several questions on their therapeutic potential in cholestasis and NASH remain open leaving a space for the development of novel compounds.
|
29649907 |
2018 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Modulation of transport and metabolism of bile acids and bilirubin by chlorogenic acid against hepatotoxicity and cholestasis in bile duct ligation rats: involvement of SIRT1-mediated deacetylation of FXR and PGC-1α.
|
29360226 |
2018 |
|
Cholestasis
|
0.600 |
AlteredExpression
|
disease |
BEFREE |
The expression of FXR target genes in the liver and intestine of cholestasis model rats was repressed.
|
30373117 |
2018 |
|
Cholestasis
|
0.600 |
Therapeutic
|
disease |
RGD |
Exploration of Hepatoprotective Effect of Gentiopicroside on Alpha-Naphthylisothiocyanate-Induced Cholestatic Liver Injury in Rats by Comprehensive Proteomic and Metabolomic Signatures.
|
30223280 |
2018 |
|
Cholestasis
|
0.600 |
Therapeutic
|
disease |
RGD |
Effects of corilagin on alleviating cholestasis via farnesoid X receptor-associated pathways in vitro and in vivo.
|
29235094 |
2018 |
|
Cholestasis
|
0.600 |
Therapeutic
|
disease |
RGD |
Taken together, 18b-GA confers hepatoprotection against ANIT-induced cholestasis by activating FXR through Sirt1, which promotes gene expression of the efflux transporter, and consequently attenuates dysregulation of bile acid homeostasis in hepatocyte compartments.
|
30061734 |
2018 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
RGD |
The ileum-liver Farnesoid X Receptor signaling axis mediates the compensatory mechanism of 17α-ethynylestradiol-induced cholestasis via increasing hepatic biosynthesis of chenodeoxycholic acids in rats.
|
30077711 |
2018 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
The findings revealed that SRT1720 treatment increased FXR and Nrf2 gene expressions to shield against hepatotoxicity and cholestasis induced by ANIT.
|
28553227 |
2017 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
In this context, therapeutic approaches including new hydrophilic BA such as the conjugation-resistant nor- ursodeoxycholic acid, nuclear receptors (FXR, PPAR-alpha) agonists, FGF19 analogues, inhibitors of the apical sodium-dependent bile acid transporter [ASBT] and modulators of the inflammatory cascade triggered by BAs are being studied as novel treatments of cholestasis.
|
29080340 |
2017 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
In this context, therapeutic approaches including the use of new hydrophilic BA such as the conjugation-resistant nor- ursodeoxycholic acid, nuclear receptor (FXR, PPAR-alpha) agonists, FGF19 analogues, inhibitors of the apical sodium-depend bile acid transporter (ASBT) and modulators of the inflammatory cascade triggered by BA are being studied as novel treatments of cholestasis.
|
31196636 |
2017 |
|
Cholestasis
|
0.600 |
Therapeutic
|
disease |
RGD |
Role of AMP-activated protein kinase α1 in 17α-ethinylestradiol-induced cholestasis in rats.
|
27090119 |
2017 |
|
Cholestasis
|
0.600 |
AlteredExpression
|
disease |
BEFREE |
In vivo, FXR knockout could significantly abrogate cholestasis induced FGF21 expression.
|
27003131 |
2016 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Clinical features of severe, persistent NR1H4-related cholestasis include neonatal onset with rapid progression to end-stage liver disease, vitamin K-independent coagulopathy, low-to-normal serum gamma-glutamyl transferase activity, elevated serum alpha-fetoprotein and undetectable liver bile salt export pump (ABCB11) expression.
|
26888176 |
2016 |
|
Cholestasis
|
0.600 |
AlteredExpression
|
disease |
BEFREE |
Hepatic nuclear receptors, VDR, HNF4α, RXRα and RARα, were induced (approximately 2.0-fold, (p<0.05) whereas FXR levels were markedly reduced to 44% of control, (p<0.05) in human obstructive cholestasis.
|
25798860 |
2015 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Cholestasis activates bile acid receptor farnesoid X receptor (FXR) and subsequently enhances hepatic expression of small heterodimer partner (SHP).
|
25943116 |
2015 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
CTD_human |
Modulation of farnesoid X receptor results in post-translational modification of poly (ADP-ribose) polymerase 1 in the liver.
|
23178280 |
2013 |
|
Cholestasis
|
0.600 |
Biomarker
|
disease |
BEFREE |
Pathways for SUMOylation were significantly altered during obstructive cholestasis with differential Sumo1 recruitment to the promoters of FXR target genes.
|
23546875 |
2013 |
|
Cholestasis
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
This suggests that a NR1H4 mutation is not or rarely involved in hepatocellular cholestasis of unknown cause.
|
23142591 |
2012 |
|
Cholestasis
|
0.600 |
GeneticVariation
|
disease |
BEFREE |
Heterozygous termination codon mutation of NR1H4 R176X was found in idiopathic infantile cholestasis.
|
21633855 |
2012 |