MYCN activation, mainly by gene amplification, is one of the most frequent molecular events in neuroblastoma (NB) oncogenesis, and is associated with increased malignancy and decreased neuronal differentiation propensity. The frequency of concomitant loss of heterozygosity at the 1p36.3 locus, which harbours the p53 anti-oncogene homologue TP73, indicates that MYCN and p73 alterations may coope...

BACKGROUND Amplification of the oncogene MYCN in neuroblastoma has been found to correlate with aggressive tumour growth and is used as a predictor of clinical outcome. The MYCN amplicon is known to involve coamplification of extensive DNA regions. Therefore it is possible that other genes are coamplified in this amplicon and that they may play a role in the poor outcome of MYCN amplified tumou...

The transcription factors of the Myb family are expressed in several tissues and play an important role in cell proliferation, differentiation, and survival In this study, the expression of A-myb, B-myb, and c-myb was investigated in a group of 64 neuroblastomas at different dinical stages by a sensitive reverse transcription-PCR tchnique and correlated with patients' survival. All of the myb g...

Amplification and enhanced expression of the MYCN oncogene are thought to contribute to the development and progression of human neuroblastomas. Here, we have transfected human neuroblastoma cells that harbor a single MYCN gene copy with the human MYCN gene driven by a viral enhancer/promoter, and we have compared the properties of the parental and the transfected cells. The transfected cells s...

PURPOSE Amplification of the MYCN proto-oncogene is strongly correlated with poor outcome in neuroblastoma (NB), although deregulated MYCN is a potent inducer of apoptosis. BIN1 (2q14) encodes multiple isoforms of a Myc-interacting adaptor protein that has features of a tumor suppressor, including the ability to inhibit Myc-mediated cell transformation and to promote apoptosis. We hypothesized ...

We developed an anti-gene peptide nucleic acid (PNA) for selective inhibition of MYCN transcription in neuroblastoma cells, targeted against a unique sequence in the antisense DNA strand of exon 2 of MYCN and linked at its NH(2) terminus to a nuclear localization signal peptide. Fluorescence microscopy showed specific nuclear delivery of the PNA in six human neuroblastoma cell lines: GI-LI-N an...

Neuroblastoma is the most common extra cranial solid tumor in childhood and the most frequently diagnosed neoplasm during the infancy. MYCN amplification and overexpression occur in about 25% of total neuroblastoma cases and this percentage increases at 30% in advanced stage neuroblastoma. So far, MYCN expression profile is still one of the most robust and significant prognostic markers for neu...

Objectives: Neuroblastoma (NB) is the most common pediatric solid tumor derived from the sympathetic nervous system. MYCN gene exists in nearly half of the NB patient and its association with rapid disease progression and poor outcome is controversial. Cancer Stem Cells (CSCs) characterization in NB has been rarely studied. This study is to figure out whether the MYCN gene and CSCs are associat...

MYCN is the product of a gene frequently deregulated in childhood tumors that belongs to a small but very famous family of transcription factors whose prototype member is c-MYC. The other member of the family is L-MYC, identified as a gene amplified in a subset of lung cancers. c-MYC is widely expressed in normal tissues, is the most deregulated protooncogene in human cancer, and not surprising...

The biologic studies of human neural crest stem cells (hNCSCs) are extremely challenging due to the limited source of hNCSCs as well as ethical and technical issues surrounding isolation of early human embryonic tissues. On the other hand, vast majority of studies on MycN have been conducted in human tumor cells, thus, the role of MycN in normal human neural crest development is completely unkn...