Search results for "N-MYC"

Gain of MYCN region in a Wilms tumor-derived xenotransplanted cell line.

2010

Wilms tumor is one of the most common pediatric malignant tumors of the kidney. Although the WT1 gene, located at 11p13, has been proven to be implicated in the development of Wilms tumor, other genes such as MYCN are also involved. The purpose of this study is to genetically characterize a Wilms tumor metastasis xenotransplanted in nude mice. Immunogenotype evolution of the xenografts material was monitored for 29 months using molecular techniques, fluorescent in situ hybridization and multiplex ligation-dependent probe amplification, in addition to immunohistochemistry in tissue microarrays. Genetic alterations present in the original tumor and retained in the xenotransplanted tumor were …

Genetic Instability and Intratumoral Heterogeneity in Neuroblastoma with MYCN Amplification Plus 11q Deletion

2013

Background/Aim Genetic analysis in neuroblastoma has identified the profound influence of MYCN amplification and 11q deletion in patients’ prognosis. These two features of high-risk neuroblastoma usually occur as mutually exclusive genetic markers, although in rare cases both are present in the same tumor. The purpose of this study was to characterize the genetic profile of these uncommon neuroblastomas harboring both these high-risk features. Methods We selected 18 neuroblastomas with MNA plus 11q loss detected by FISH. Chromosomal aberrations were analyzed using Multiplex Ligation-dependent Probe Amplification and Single Nucleotide Polymorphism array techniques. Results and Conclusion Thi…

MBP-1 represses N-MYC expression and acts as a tumor suppressor in human Neuroblastoma LAN-5 cells

2013

Neuroblastoma is the most common extra-cranial solid tumor of childhood, originated from cells of the neural crest. Amplification of N-MYC gene and 1p-deletion are found in more than 30% of patients with advanced stages and they are associated with poor prognosis. An alternative translated product of the ENO1 gene, known as MBP-1 (c-myc promoter binding protein-1), acts as a negative regulator of the c-MYC oncogene, ERBB2 and COX-2 genes, furthermore, ENO1/MBP-1 overexpression in Neuroblastoma cells significantly reduces cell growth and induces apoptosis. Even though there are similarities between the c-MYC and N-MYC oncogenes, there are no evidences that MBP-1 is able to interact with N-MY…

MBP-1 reprime l’espressione di N-MYC e svolge il ruolo di oncosoppressore in cellule di Neuroblastoma umano LAN5

2013

Il Neuroblastoma, derivato da cellule neurali simpatiche primitive, è il tumore solido extracranico più comune dell'infanzia. L'amplificazione del gene N-MYC insieme a delezioni nel cromosoma 1p36, sono i marcatori molecolari più frequenti nel Neuroblastoma e sono associati a cattiva prognosi. MBP-1, prodotto alternativo della traduzione dell'mRNA del gene ENO1, è un repressore trascrizionale e agisce direttamente sul promotore dei geni c-MYC, ERBB2 e COX2 (1-3). In cellule di Neuroblastoma è stato osservato che l'espressione ectopica di ENO1/MBP-1 causa induzione di apoptosi e morte cellulare (4). Sebbene esistano similarità di struttura e funzionali tra i geni N-MYC e c-MYC non è noto se …

Identification of a novel recurrent 1q42.2-1qter deletion in high risk MYCN single copy 11q deleted neuroblastomas

2012

Neuroblastoma is an aggressive embryonal tumor that accounts for similar to 15% of childhood cancer deaths. Hitherto, despite the availability of comprehensive genomic data on DNA copy number changes in neuroblastoma, relatively little is known about the genes driving neuroblastoma tumorigenesis. In this study, high resolution array comparative genome hybridization (CGH) was performed on 188 primary neuroblastoma tumors and 33 neuroblastoma cell lines to search for previously undetected recurrent DNA copy number gains and losses. A new recurrent distal chromosome 1q deletion (del(1)(q42.2qter)) was detected in seven cases. Further analysis of available array CGH datasets revealed 13 additio…

Prognostic value of SOX2 expression in neuroblastoma.

2010

Circulating MYCN DNA Predicts MYCN-Amplification in Neuroblastoma

2005

Age Dependency of the Prognostic Impact of Tumor Genomics in Localized Resectable MYCN-Nonamplified Neuroblastomas. Report From the SIOPEN Biology Gr…

2020

Purpose: For localized, resectable neuroblastoma without MYCN amplification, surgery only is recommended even if incomplete. However, it is not known whether the genomic background of these tumors may influence outcome. Patients and methods: Diagnostic samples were obtained from 317 tumors, International Neuroblastoma Staging System stages 1/2A/2B, from 3 cohorts: Localized Neuroblastoma European Study Group I/II and Children's Oncology Group. Genomic data were analyzed using multi- and pangenomic techniques and fluorescence in-situ hybridization in 2 age groups (cutoff age, 18 months) and were quality controlled by the International Society of Pediatric Oncology European Neuroblastoma (SIO…

Segmental chromosomal alterations have prognostic impact in neuroblastoma: a report from the INRG project

2012

Background: In the INRG dataset, the hypothesis that any segmental chromosomal alteration might be of prognostic impact in neuroblastoma without MYCN amplification (MNA) was tested. Methods: The presence of any segmental chromosomal alteration (chromosome 1p deletion, 11q deletion and/or chromosome 17q gain) defined a segmental genomic profile. Only tumours with a confirmed unaltered status for all three chromosome arms were considered as having no segmental chromosomal alterations. Results: Among the 8800 patients in the INRG database, a genomic type could be attributed for 505 patients without MNA: 397 cases had a segmental genomic type, whereas 108 cases had an absence of any segmental a…

Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblasto…

2011

BACKGROUND: In neuroblastoma (NB), the presence of segmental chromosome alterations (SCAs) is associated with a higher risk of relapse. METHODS: In order to analyse the role of SCAs in infants with localised unresectable/disseminated NB without MYCN amplification, we have performed an array CGH analysis of tumours from infants enrolled in the prospective European INES trials. RESULTS: Tumour samples from 218 out of 300 enroled patients could be analysed. Segmental chromosome alterations were observed in 11%, 20% and 59% of infants enroled in trials INES99.1 (localised unresectable NB), INES99.2 (stage 4s) and INES99.3 (stage 4) (P<0.0001). Progression-free survival was poorer in patients wh…