A novel transcriptional suppressor located within a downstream intron of the BCR gene

J Biol Chem. 1994 Apr 8;269(14):10820-9.

Abstract

A reciprocal translocation between chromosomes 9 and 22 creates the Philadelphia (Ph1) chromosome in chronic myelogenous leukemia. This translocation results in the fusion of the ABL and the BCR genes to form a BCR/ABL fusion gene, the product of which has a greatly increased protein tyrosine kinase activity in comparison with the normal ABL protein. The chromosome 22 translocation breakpoints are concentrated within a 5.8-kilobase region named the major break-point cluster region (Mbcr). Gel mobility shift and DNase I footprinting assays have defined binding sites for three proteins, BIF 1-3 (BCR intron factors 1-3), lying within a 427-base pair fragment of the Mbcr. This 427-base pair fragment functions as a transcriptional silencer with both the BCR as well as a heterologous promoter. The silencing is position- and orientation-independent. The transcriptional effects are greatest in chronic myelogenous leukemia cells, decreased in HeLa and B-cells, and absent in T-lymphocytes. Gel mobility shift assays show a corresponding difference in pattern when the T-lymphocyte nuclear extract is compared with other cell lines. The Mbcr appears to contain a novel group of transcriptional silencers that share a common binding motif with a recently described suppressor in the mouse Adh-1 gene.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Base Composition
  • Base Sequence
  • Binding Sites
  • Cell Line
  • DNA / metabolism
  • Fusion Proteins, bcr-abl / genetics
  • Gene Expression Regulation
  • HeLa Cells
  • Humans
  • Introns*
  • Molecular Sequence Data
  • Mutation
  • Promoter Regions, Genetic
  • Protein-Tyrosine Kinases*
  • Proto-Oncogene Proteins / genetics*
  • Proto-Oncogene Proteins c-bcr
  • Regulatory Sequences, Nucleic Acid*
  • Transcription, Genetic*

Substances

  • Proto-Oncogene Proteins
  • DNA
  • Protein-Tyrosine Kinases
  • Fusion Proteins, bcr-abl
  • BCR protein, human
  • Proto-Oncogene Proteins c-bcr

Associated data

  • GENBANK/M55395