SHH, sonic hedgehog signaling molecule, 6469

N. diseases: 303; N. variants: 44
Disease: Childhood Medulloblastoma ×
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Analysis of PTCH/SMO/SHH pathway genes in medulloblastoma. 10564585 2000
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 GeneticVariation disease BEFREE In contrast, mice lacking one or two functional Ink4c alleles and one copy of Patched (Ptc1) encoding the Shh receptor rapidly developed MBs that retained wild-type p53. 16260494 2005
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE We therefore reason that targeted inhibition of miR-182 may prevent leptomeningeal spread in patients with non-SHH-MB. 22134538 2012
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Furthermore, based on the enrichment of MYCN and GLI2 amplifications in SHH-driven medulloblastoma, amplification of these downstream signaling intermediates should be taken into account before a patient is enrolled into a clinical trial using a smoothened inhibitor. 22160402 2012
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE CGNPs are the cells of origin for SHH-driven medulloblastoma, the most common malignant brain tumor in children. 22302101 2012
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 AlteredExpression disease BEFREE Canonical TGF-β pathway activity is a predictor of SHH-driven medulloblastoma survival and delineates putative precursors in cerebellar development. 22966790 2013
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Medulloblastoma (MB) is a malignant pediatric brain tumor arising in the cerebellum consisting of four distinct subgroups: WNT, SHH, Group 3 and Group 4, which exhibit different molecular phenotypes. 23567267 2013
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 GeneticVariation disease BEFREE TERT mutations define a subset of SHH medulloblastoma with distinct demographics, cytogenetics, and outcomes. 24174164 2013
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE The two most well-defined subgroups are caused by overactive signaling in the WNT and SHH mitogenic pathways; less is understood about Groups 3 and 4 medulloblastoma. 24252460 2013
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 GeneticVariation disease BEFREE Recent molecular profiling has suggested the stratification of medulloblastoma from one single disease into four distinct subgroups namely: WNT Group (best prognosis), SHH Group (intermediate prognosis), Group 3 (worst prognosis) and Group 4 (intermediate prognosis). 24460684 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 GeneticVariation disease BEFREE Genome sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition. 24651015 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Our study showed postoperative primary CHT significantly influence the survival of CMB, SHH subgroup and WNT subgroup but not in DMB and Non-SHH/WNT subgroup of MB. 24932704 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE These processes are poorly understood in medulloblastoma (MB), in which diverse oncogenic pathways define at least four molecular disease subtypes (WNT, SHH, Group 3 and Group 4). 25163932 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Although the Shh-binding protein Boc associates with the Shh receptor Ptch1 to mediate Shh signaling, whether Boc plays a role in medulloblastoma is unknown. 25263791 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Prospective studies have documented the efficacy of SMO inhibitors in a subgroup of patients with SHH medulloblastoma. 25398846 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Clustering, co-expression network, and gene expression analyses revealed that WNT and SHH medulloblastoma may be derived from distinct neural stem cell populations during early embryonic development, while the transcriptional profiles of Group 3 and Group 4 medulloblastoma resemble cerebellar granule neuron precursors at weeks 10-15 and 20-30 of embryogenesis, respectively. 25412507 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Children with WNT/TP53 mutant medulloblastoma had higher 5-year survival than those with SHH/TP53 mutant tumours (100% and 36.6%±8.7%, respectively (p<0.001)). 25539912 2014
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Contrast enhancement pattern predicts poor survival for patients with non-WNT/SHH medulloblastoma tumours. 25862008 2015
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Here, we employed a high throughput flow cytometry screen to identify differentially expressed cell surface markers in self-renewing vs. non-self-renewing SHH medulloblastoma cells. 26497209 2015
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Thus, cell senescence is an important characteristic of a subset of SHH medulloblastoma and might explain the acquisition of somatic TP53 mutations in human medulloblastoma. 27229128 2016
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 GeneticVariation disease BEFREE In this context our investigation documents that Vandetanib in combination with the clinically available PI3K inhibitor GDC-0941 leads to enhanced cytotoxicity against MYC-amplified and SHH-TP53-mutated medulloblastoma. 28159923 2017
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 AlteredExpression disease BEFREE Among the four different medulloblastoma subgroups described to date, the sonic hedgehog (SHH) genetic pathway is the pathway activated in the tumorigenesis of medulloblastoma. 28218785 2017
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Selective targeting of HDAC1/2 elicits anticancer effects through Gli1 acetylation in preclinical models of SHH Medulloblastoma. 28276480 2017
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 Biomarker disease BEFREE Conversely, <i>ATOH1</i> expression was detected consistently in recurrent and metastatic SHH medulloblastoma. 28490517 2017
CUI: C0278510
Disease: Childhood Medulloblastoma
Childhood Medulloblastoma 		0.100 GeneticVariation disease BEFREE High TrkC mRNA expression appears to be frequent in the SHH subgroup and seems not to have a major effect on therapy responsiveness in medulloblastoma patients. 28695340 2017