|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Here we use a mouse model of Ptch1 heterozygosity to reveal a critical tumor suppressor function for Hic1 in medulloblastoma.
|
18347096 |
2008 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Finally, compound 24 led to significant regression of subcutaneous tumor generated by primary Ptch1-deficient medulloblastoma cells in SCID mouse.
|
28618224 |
2017 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Medulloblastoma and RMS are also present in the murine model for Ptch1 deficiency.
|
12845631 |
2003 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Together, our results indicate that DNA damage promoted by Boc leads to the demise of its own coreceptor, Ptch1, and consequently medulloblastoma progression.
|
25263791 |
2014 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
We firstly isolated CSCs from Sonic Hedgehog (SHH) MB derived from Ptch1 heterozygous mice and compared their expression level of EMT-related transcripts and microRNAs with cerebellar NSCs.
|
30483126 |
2018 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Orthotopic transplantation of NES cells generated from Gorlin syndrome patients, who are predisposed to medulloblastoma due to germline-mutated PTCH1, also generated medulloblastoma.
|
31204176 |
2019 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Recent advances in the molecular genetics of medulloblastoma transformation (e.g., myc, PTCH ) are reviewed and discussed.
|
11772307 |
2001 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
If one copy of the Ptch gene is lost, as in human Gorlin's syndrome and in Ptch(+/-) mice, MBs may form.
|
23389290 |
2013 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Surprisingly, GADD34 homozygous mutation strongly enhanced the ISR, but significantly decreased the incidence of medulloblastoma in adult Ptch1+/- mice.
|
27802424 |
2016 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Shh-associated MBs originate postnatally, from dysregulated hyperproliferation of GCPs in developing cerebellum's external granular layer (EGL), as shown in heterozygous Ptch1<sup>+/-</sup> knock-out mouse strains that model human MB occurrence and progression.
|
30452905 |
2019 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Here we have used Ptc1(+/-); p53(-/-) mice which develop medulloblastoma to test the ability of cyclopamine to inhibit endogenous tumor growth in vivo after tumor initiation through intraperitoneal delivery, which avoids the brain damage associated with direct injection.
|
15652709 |
2005 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
To study the role of Ptc1 in cerebellar tumor development and to create a preclinical therapeutic platform, we have generated a conditional Ptc1 haploinsufficiency model of medulloblastoma by inactivating Ptc1 in Pax7-expressing cells of the cerebellum.
|
19213072 |
2009 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
CTD_human |
To study the role of Ptc1 in cerebellar tumor development and to create a preclinical therapeutic platform, we have generated a conditional Ptc1 haploinsufficiency model of medulloblastoma by inactivating Ptc1 in Pax7-expressing cells of the cerebellum.
|
19213072 |
2009 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Mutations in the human tumor suppressor gene, Patched-1, are associated with nevoid basal cell carcinoma syndrome characterized by developmental abnormalities and tumorigenesis, such as basal cell carcinoma and medulloblastoma.
|
16934747 |
2006 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Treatment of Med in Ptch1(+/-) Trp53(-/-) mice with the antiestrogen chemotherapeutic drug Faslodex significantly increased symptom-free survival, which was associated with increased apoptosis and decreased BCL2 and IGF1R expression and signaling.
|
25885794 |
2015 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
In conjunction with CD15, proliferating CD24+/CD15+ granule cell precursors (GCPs) were identified as a TIC population in Ptch1 deleted medulloblastoma.
|
30657775 |
2019 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Most human MB cell lines tested (five of seven = 71.4%), two MB cell lines derived from spontaneously arising tumors in Patched-1(+/-) mice (two of two = 100%) and three MB primary cultures derived from surgical specimens, were susceptible to reovirus infection.
|
12810644 |
2003 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Primary tumor cells derived from two models of murine medulloblastoma (Ptch1(+/-);Ink4c(-/-) and p53(FL/FL);Nestin-Cre(+); Ink4c(-/-)) that retain and lack p53 function, respectively, displayed a dependence on functional p53 to engage 17-DMAG-induced apoptosis.
|
19805107 |
2009 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
CTD_human |
When compared with Ptch1 heterozygous mutants, compound Ptch1/Hic1 heterozygotes display a fourfold increased incidence of medulloblastoma.
|
18347096 |
2008 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Learning that Ptc1 is a medulloblastoma tumor suppressor led directly to the identification of the Ptc1 ligand, Sonic hedgehog, as a powerful mitogen for cerebellar granule cell precursors.
|
11283316 |
2001 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
Mice heterozygous for Patched-1 (Ptc1+/-) that are either heterozygous or nullizygous for Kip1 developed medulloblastoma rapidly and with high penetrance.
|
19147535 |
2009 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
There is a strong association between anaplastic/large-cell tumours and MYC amplification, which has previously been linked with aggressive disease, but associations between abnormalities on chromosome 17 and anaplastic/large-cell MBs and between abnormalities in the shh/PTCH pathway and the desmoplastic variant are more controversial.
|
12175339 |
2002 |
|
Childhood Medulloblastoma
|
0.400 |
Biomarker
|
disease |
BEFREE |
To investigate the mechanisms of genetic interactions between Shh and IGF signaling in the cerebellum, we crossed nestin/IGF-I transgenic (IGF-I Tg) mice, in which transgene expression occurs in neuron precursors, with Ptc1+/- knockout mice, a model of medulloblastoma in which cancer develops in a multistage process.
|
20214787 |
2010 |
|
Childhood Medulloblastoma
|
0.400 |
PosttranslationalModification
|
disease |
BEFREE |
Whereas genetic mutations in PTCH1 have previously been shown to lead to medulloblastoma, our study indicates that epigenetic silencing of PTCH1, and other critical developmental loci, by DNA methylation is a fundamental process of pediatric medulloblastoma formation.
|
19966297 |
2010 |
|
Childhood Medulloblastoma
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Constitutive activation of hedgehog signaling, often caused by PTCH1 inactivation and leading to inappropriate activation of GLI target genes, is crucial for the development of several human tumors including basal cell carcinoma of the skin and medulloblastoma.
|
15521068 |
2005 |