The role of CALM-AF10 gene fusion in acute leukemia

Leukemia. 2008 Apr;22(4):678-85. doi: 10.1038/sj.leu.2405074. Epub 2007 Dec 20.

Abstract

Chromosomal translocations are important genetic perturbations frequently associated with hematologic malignancies; characterization of these events has been a rich source of insights into the mechanisms that lead to malignant transformation. The t(10;11)(p13;q14-21) results in a recently identified rare but recurring chromosomal translocation seen in patients with ALL as well as AML, and results in the production of a CALM-AF10 fusion gene. Although the details by which the CALM-AF10 fusion protein exerts its leukemogenic effect remain unclear, emerging data suggests that the CALM-AF10 fusion impairs differentiation of hematopoietic cells, at least in part via an upregulation of HOXA cluster genes. This review discusses the normal structure and function of CALM and AF10, describes the spectrum of clinical findings seen in patients with CALM-AF10 fusions, summarizes recently published CALM-AF10 mouse models and highlights the role of HOXA cluster gene activation in CALM-AF10 leukemia.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Acute Disease
  • Chromosomes, Human, Pair 10
  • Chromosomes, Human, Pair 11
  • Gene Fusion*
  • Homeodomain Proteins / genetics
  • Homeodomain Proteins / physiology
  • Humans
  • Leukemia / genetics*
  • Oncogene Proteins, Fusion / physiology*
  • Translocation, Genetic

Substances

  • AF10-CALM fusion protein, human
  • Homeodomain Proteins
  • Oncogene Proteins, Fusion
  • HoxA protein