E proteins in lymphocyte development and lymphoid diseases

Curr Top Dev Biol. 2014;110:153-87. doi: 10.1016/B978-0-12-405943-6.00004-X.


As members of the basic helix-loop-helix (bHLH) family of transcription factors, E proteins function in the immune system by directing and maintaining a vast transcriptional network that regulates cell survival, proliferation, differentiation, and function. Proper activity of this network is essential to the functionality of the immune system. Aberrations in E protein expression or function can cause numerous defects, ranging from impaired lymphocyte development and immunodeficiency to aberrant function, cancer, and autoimmunity. Additionally, disruption of inhibitor of DNA-binding (Id) proteins, natural inhibitors of E proteins, can induce additional defects in development and function. Although E proteins have been investigated for several decades, their study continues to yield novel and exciting insights into the workings of the immune system. The goal of this chapter is to discuss the various classical roles of E proteins in lymphocyte development and highlight new and ongoing research into how these roles, if compromised, can lead to disease.

Keywords: B cell; Burkitt Lymphoma; Development; E2A; HEB; Id2; Id3; Sjogren's Syndrome; T cell.

Publication types

  • Review

MeSH terms

  • Animals
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / metabolism
  • Autoimmune Diseases / pathology
  • Basic Helix-Loop-Helix Transcription Factors / chemistry
  • Basic Helix-Loop-Helix Transcription Factors / genetics
  • Basic Helix-Loop-Helix Transcription Factors / metabolism*
  • Cell Differentiation
  • Cell Survival
  • Humans
  • Lymphatic Diseases / genetics
  • Lymphatic Diseases / metabolism*
  • Lymphatic Diseases / pathology
  • Lymphocytes / cytology
  • Lymphocytes / physiology*
  • Neoplasms / genetics
  • Neoplasms / metabolism
  • Neoplasms / pathology
  • Receptors, Antigen / metabolism


  • Basic Helix-Loop-Helix Transcription Factors
  • Receptors, Antigen
  • TCF3 protein, human
  • TCF12 protein, human