Histone deacetylase 1 and 2 are essential for normal T-cell development and genomic stability in mice

Blood. 2013 Feb 21;121(8):1335-44. doi: 10.1182/blood-2012-07-441949. Epub 2013 Jan 3.

Abstract

Histone deacetylase 1 and 2 (HDAC1/2) regulate chromatin structure as the catalytic core of the Sin3A, NuRD and CoREST co-repressor complexes. To better understand the key pathways regulated by HDAC1/2 in the adaptive immune system and inform their exploitation as drug targets, we have generated mice with a T-cell specific deletion. Loss of either HDAC1 or HDAC2 alone has little effect, while dual inactivation results in a 5-fold reduction in thymocyte cellularity, accompanied by developmental arrest at the double-negative to double-positive transition. Transcriptome analysis revealed 892 misregulated genes in Hdac1/2 knock-out thymocytes, including down-regulation of LAT, Themis and Itk, key components of the T-cell receptor (TCR) signaling pathway. Down-regulation of these genes suggests a model in which HDAC1/2 deficiency results in defective propagation of TCR signaling, thus blocking development. Furthermore, mice with reduced HDAC1/2 activity (Hdac1 deleted and a single Hdac2 allele) develop a lethal pathology by 3-months of age, caused by neoplastic transformation of immature T cells in the thymus. Tumor cells become aneuploid, express increased levels of c-Myc and show elevated levels of the DNA damage marker, γH2AX. These data demonstrate a crucial role for HDAC1/2 in T-cell development and the maintenance of genomic stability.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Animals, Newborn
  • Cell Transformation, Neoplastic / genetics*
  • Cell Transformation, Neoplastic / immunology
  • Chromatin / genetics
  • Chromosome Aberrations
  • DNA Damage / genetics
  • DNA Damage / immunology
  • Enzyme Activation / genetics
  • Enzyme Activation / immunology
  • Female
  • Genomic Instability / genetics*
  • Genomic Instability / immunology
  • Haploinsufficiency / genetics
  • Haploinsufficiency / immunology
  • Histone Deacetylase 1 / genetics*
  • Histone Deacetylase 1 / metabolism
  • Histone Deacetylase 2 / genetics*
  • Histone Deacetylase 2 / metabolism
  • Male
  • Mice
  • Mice, Knockout
  • Signal Transduction / genetics
  • Signal Transduction / immunology
  • T-Lymphocytes / cytology
  • T-Lymphocytes / enzymology*
  • Thymus Gland / cytology
  • Transcriptome / immunology

Substances

  • Chromatin
  • Hdac1 protein, mouse
  • Hdac2 protein, mouse
  • Histone Deacetylase 1
  • Histone Deacetylase 2