Lack of the ubiquitin-editing enzyme A20 results in loss of hematopoietic stem cell quiescence

J Exp Med. 2015 Feb 9;212(2):203-16. doi: 10.1084/jem.20132544. Epub 2015 Jan 26.

Abstract

A balance between quiescence and proliferation is critical for proper maintenance of the hematopoietic stem cell (HSC) pool. Although a lot is known about hematopoiesis, molecular mechanisms that control HSC quiescence remain largely unknown. The ubiquitin-editing enzyme A20 functions as a central regulator of inflammation and adaptive immunity. Here, we show that a deficiency of A20 in the hematopoietic system causes anemia, lymphopenia, and postnatal lethality. Lack of A20 in HSCs results in diminished pool size, impaired radioprotection, defective repopulation, and loss of quiescence. A20-deficient HSCs display increased IFN-γ signaling, caused by augmented NF-κB activation. Strikingly, deletion of both IFN-γ and A20 in hematopoietic cells results in partial rescue of the HSC phenotype. We anticipate that our experiments will facilitate the understanding of mechanisms through which A20-mediated inflammatory signals control HSC quiescence and functions.

MeSH terms

  • Animals
  • Cell Cycle / genetics*
  • Cysteine Endopeptidases / deficiency*
  • Cysteine Endopeptidases / genetics
  • Female
  • Genes, Lethal
  • Genetic Association Studies
  • Hematopoiesis / genetics
  • Hematopoietic Stem Cells / cytology*
  • Hematopoietic Stem Cells / metabolism*
  • Interferon-gamma / genetics
  • Interferon-gamma / metabolism
  • Intracellular Signaling Peptides and Proteins / deficiency*
  • Intracellular Signaling Peptides and Proteins / genetics
  • Mice
  • Mice, Knockout
  • Mutation
  • NF-kappa B / metabolism
  • Phenotype
  • Signal Transduction
  • Tumor Necrosis Factor alpha-Induced Protein 3
  • Ubiquitination / genetics*

Substances

  • Intracellular Signaling Peptides and Proteins
  • NF-kappa B
  • Interferon-gamma
  • Tumor Necrosis Factor alpha-Induced Protein 3
  • Cysteine Endopeptidases
  • Tnfaip3 protein, mouse