Chimaeric oncoproteins resulting from chromosomal translocations in acute lymphoblastic leukaemia

Semin Cancer Biol. 1993 Dec;4(6):387-99.

Abstract

A major mode of proto-oncogene activation by chromosomal translocations concerns the creation of fusion genes which encode chimaeric proteins. The largest class of oncogenes identified to date is the transcription factors, which are involved in control of cellular proliferation and differentiation via regulation of target gene transcription. Protein chimaeras that result from translocations in childhood ALL include two which involve the 19p13.3 gene E2A and a large heterogeneous group involving HRX located at chromosome band 11q23. Functional studies demonstrate that E2A fusion proteins function as chimaeric transcription factors, and structural features suggest that HRX fusion proteins may have analogous properties.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Base Sequence
  • Basic-Leucine Zipper Transcription Factors
  • Cell Transformation, Neoplastic / genetics
  • Chromosomes, Human, Pair 1 / ultrastructure*
  • Chromosomes, Human, Pair 17 / ultrastructure
  • Chromosomes, Human, Pair 19 / ultrastructure*
  • Consensus Sequence
  • DNA-Binding Proteins / genetics*
  • DNA-Binding Proteins / physiology
  • Drosophila Proteins*
  • Drosophila melanogaster / genetics
  • Gene Expression Regulation, Leukemic
  • Helix-Loop-Helix Motifs / genetics
  • Humans
  • Leukemia, Experimental / genetics
  • Mice
  • Mice, Nude
  • Mice, Transgenic
  • Models, Genetic
  • Molecular Sequence Data
  • Multigene Family
  • Oncogene Proteins, Fusion / genetics*
  • Oncogene Proteins, Fusion / physiology
  • Oncogenes*
  • Pre-B-Cell Leukemia Transcription Factor 1
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • Proto-Oncogene Proteins / genetics*
  • Proto-Oncogene Proteins / physiology
  • Sequence Alignment
  • Sequence Homology, Nucleic Acid
  • TCF Transcription Factors
  • Transcription Factor 7-Like 1 Protein
  • Transcription Factors*
  • Translocation, Genetic*
  • Zinc Fingers / genetics

Substances

  • Basic-Leucine Zipper Transcription Factors
  • DNA-Binding Proteins
  • Drosophila Proteins
  • E2a-Hlf fusion protein, human
  • E2a-Hlf fusion protein, mouse
  • Oncogene Proteins, Fusion
  • Pre-B-Cell Leukemia Transcription Factor 1
  • Proto-Oncogene Proteins
  • TCF Transcription Factors
  • TCF7L1 protein, human
  • Tcf7l1 protein, mouse
  • Transcription Factor 7-Like 1 Protein
  • Transcription Factors
  • Trl protein, Drosophila
  • pbx1 protein, human