Runx1/AML1 in leukemia: disrupted association with diverse protein partners

Leuk Res. 2002 Mar;26(3):221-8. doi: 10.1016/s0145-2126(01)00128-x.

Abstract

Runx1/AML1, a chromosome 21q22 hematopoietic regulator, is frequently translocated in leukemia. Its protein product, a relatively weak transcriptional activator, becomes an effective transcriptional enhancer or repressor, when co-operating with transcriptional co-activators or co-repressors. Runx1/AML1 association with its partners is disrupted in leukemia. For example, Runx1/AML1 mutations and translocations (e.g. t(8;21), t(12;21) and t(3;21)) impair binding of Runx1/AML1-CBFbeta complexes to Runt motifs in myelopoietically active promoters, preventing normal hematopoiesis. However, Runx1/AML1-associated translocations are not leukemogenic in animal models, suggesting the involvement of yet unidentified regulatory proteins. New candidates are cholinesterases, inhibition of which increases leukemic risk in a manner potentially associated with Runx1/AML1.

Publication types

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

MeSH terms

  • Chromosome Mapping
  • Chromosomes, Human, Pair 21
  • Chromosomes, Human, Pair 8
  • Core Binding Factor Alpha 2 Subunit
  • DNA-Binding Proteins / analysis
  • DNA-Binding Proteins / genetics*
  • Hematopoiesis
  • Humans
  • Leukemia / genetics*
  • Neoplasm Proteins / genetics
  • Point Mutation
  • Proto-Oncogene Proteins*
  • Transcription Factors / analysis
  • Transcription Factors / genetics*
  • Transcription, Genetic
  • Transcriptional Activation
  • Translocation, Genetic*

Substances

  • Core Binding Factor Alpha 2 Subunit
  • DNA-Binding Proteins
  • Neoplasm Proteins
  • Proto-Oncogene Proteins
  • RUNX1 protein, human
  • Transcription Factors