LOCALIZATION OF MBP1/a-ENOLASE GENE TO CHROMOSOME 1p36.2-3 AND ALTERATIONS OF THIS GENE IN CHILDHOOD NEUROBLASTOMA

Kong X-T, Wilkinson DA, Valentine MB, Ragsdale ST, Valentine VA, Rowe ST, and Look AT

Department of Experimental Oncology, St Jude Children's Research Hospital, Memphis, TN, USA

 

Objective: To determine whether inactivation of MBP-1/a-enolase, a gene encoding C-myc promoter binding protein, contribute to the genesis or progression of childhood neuroblastoma with MYCN amplification.

Methods: Fluorescence in situ hybridization (FISH) analysis

Results: Eighteen neuroblastoma cell lines were examined by FISH analysis. We found that the MBP-1/a-enolase gene was assigned to chromosome bands 1p36.2-3, which is included in the large regions deleted very frequently in neuroblastoma with MYCN amplifications. Seventeen of the 18 lines showed deletion or translocation of one allele of the MBP-1/a-enolase gene on chromosome 1, suggesting that the MBP-1/a-enolase gene locus was consistently affected in neuroblastoma cells, probably contributing to the inactivation of this gene in neuroblastomas. However, sequence analysis of the cell lines only remaining one allele of the gene, did not show any inactivating mutations, suggesting that the MBP-1/a-enolase gene may be not homozygously inactivated in this tumor. Other mechanisms, together with the deletion or translocation of the one allele of the MBP-1/a-enolase gene may contribute to the genesis or progression of neuroblastoma.

Conclusion: Consistent affecting of MBP-1/a-enolase gene in most neuroblastoma cell lines with MYCN amplification found in this study suggest that MBP-1/a-enolase gene plays an important role in the pathogenesis of neuroblastomas with MYCN amplification.

 

 
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