|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
LHGDN |
Deregulation of the APC/beta-catenin pathway occurs in a consistent fraction of hepatoblastomas, with mutations in the APC and beta-catenin genes implicated in familial adenomatous polyposis-associated and sporadic hepatoblastomas, respectively.
|
17962810 |
2008 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
This study confirms that beta-catenin deregulation is involved in sporadic hepatoblastoma and also suggests that mismatch repair defects and p53 mutations contribute to this rare liver cancer.
|
17962810 |
2008 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Among human cancers tightly linked to abnormal Wnt/β-catenin signaling, hepatoblastoma (HB) presents with the highest rate (50-90%) of β-catenin mutations.
|
19646548 |
2011 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
In CTNNB1-mutated hepatoblastoma, expression of GS was only detected in tumour areas with epithelial, not with mesenchymal differentiation.
|
21237236 |
2011 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
CTD_human |
Differential expression of glutamine synthetase and cytochrome P450 isoforms in human hepatoblastoma.
|
21237236 |
2011 |
|
Hepatoblastoma
|
0.700 |
PosttranslationalModification
|
disease |
BEFREE |
Our results also revealed a large subset of HB, 83%, with cytoplasmic expression of tyrosine654-phosphorylated beta-catenin and 30% showing additional nuclear accumulation.
|
21992464 |
2011 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Tumors derived from 56 HBL cases treated with the Japanese Study Group for Pediatric Liver Tumors (JPLT) Protocol-2 were analyzed for oncogenic mutations (missense mutations and interstitial deletions in the third exon) of the CTNNB1 gene-encoding β-catenin and for the expression levels of telomerase reverse transcriptase (TERT).
|
22152854 |
2011 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
A further investigation using N- and C-terminal-specific β-catenin antibodies on human hepatoblastomas revealed a correlation between full-length versus truncated β-catenin and differentiation status, with embryonal hepatoblastomas expressing full-length β-catenin and fetal hepatoblastomas expressing β-catenin lacking its N terminus.
|
22613727 |
2012 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
In conclusion, our data clearly identify protein stabilizing mutations of the β-catenin gene as a common feature of nested stromal epithelial tumors of the liver, similarly as in hepatoblastomas.
|
22749188 |
2012 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
The current observation of a somatic CTNNB1 mutation in a hepatoblastoma from a patient with a germline GPC3 mutation supports the notion that the mutation in GPC3 may influence one of the initial steps in tumorigenesis and the progression to hepatoblastoma.
|
24459012 |
2014 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Small interfering RNA-mediated knockdown of Yap1 or β-catenin in hepatoblastoma cells reduced proliferation in an additive manner.
|
24837480 |
2014 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
These findings suggested the activation of the Wnt pathway in HB, which was confirmed by immunohistochemical staining of the β-catenin in 42 HB tumors.
|
24912477 |
2014 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Whole-exome sequencing identified HB as a genetically very simple tumour (2.9 mutations per tumour) with recurrent mutations in ß-catenin (CTNNB1) (12/15 cases) and the transcription factor NFE2L2 (2/15 cases).
|
25135868 |
2014 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
By contrast, the expression levels of epithelial-cadherin (E-cadherin) and cytosolic accumulation of β-catenin, the two most prominent markers involved in epithelial-mesenchymal transition (EMT), were reduced in liver specimens from patients with metastatic HB compared with that of healthy adjacent control tissue.
|
25695679 |
2015 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
We transfected the parental HuH6 hepatoblastoma cell line with a doxycycline-inducible shRNA against CTNNB1 (gene coding for β-catenin) to obtain an isogenic cell line pair with or without aberrant β-catenin signaling.
|
26715116 |
2015 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
To address their roles in the pathogenesis of HB, we generated mice in which Myc and mutant β-catenin were targeted to immature cells of the developing mouse liver.
|
27734029 |
2016 |
|
Hepatoblastoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
However, variable survival rates of 60-80% and debilitating chemotherapy sequelae argue for more informed treatment selection, which is not possible by grading the Wnt-β-catenin over activity present in most HBL tumors.
|
27910913 |
2016 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
We detected nuclear β-catenin immunostaining in nearly all untreated HBs, including in fetal and embryonal epithelial components and in mesenchymal elements.
|
28277286 |
2017 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Nuclear β-catenin staining which is significantly common in embryonal elements in HB predicts shorter survival.
|
28631020 |
2017 |
|
Hepatoblastoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Nuclear β-catenin expression was significantly associated with membranous epithelial cell adhesion molecule (EpCAM) expression in hepatoblastoma tumor specimens.
|
28851352 |
2017 |
|
Hepatoblastoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
All 3 (100%) mixed epithelial and mesenchymal HBL harbored CTNNB1 mutation.
|
29079173 |
2017 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Recent studies suggest that activation of Yes-associated protein (YAP) is a major molecular event in HB development, as activated YAP synergizes with mutant β-catenin to promote HB formation in mice (YAP/β-catenin).
|
29088718 |
2017 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
The Wnt/β-catenin pathway plays a central role in the pathogenesis of most hepatoblastomas (HBs), that is, up to 60-80% carry activating CTNNB1 mutations.
|
29446530 |
2018 |
|
Hepatoblastoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Also, niclosamide, a Food and Drug Administration approved antihelminth compound, could effectively inhibit HB cell growth in vitro and in vivo via downregulation of Dvl-2 and β-catenin expression.
|
29528187 |
2018 |
|
Hepatoblastoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Here, we identify lipocalin 2 (Lcn2) as a target of β-catenin and YAP1 in HB and show that serum Lcn2 values positively correlated with tumor burden.
|
29920228 |
2018 |