title: Altered expression of the TCR signaling related genes CD3 and FcεRIγ in patients with aplastic anemia.
abstract: BACKGROUND
  Aplastic anemia (AA) is characterized by pancytopenia and bone marrow hypoplasia, which results from immune-mediated hematopoiesis suppression. Understanding the pathophysiology of the immune system, particularly T cells immunity, has led to improved AA treatment over the past decades. However, primary and secondary failure after immunosuppressive therapy is frequent. Thus, knowledge of the immune mechanisms leading to AA is crucial to fundamentally understand the disease.
  
  
  RESULTS
  To elucidate the T cell receptor (TCR) signal transduction features in AA, the expression levels of CD3γ, δ, ε and ζ chain and FcεRIγ genes, which are involved in TCR signal transduction, and the negative correlation of the expression levels between the CD3ζ and FcεRIγ genes in T cells from peripheral blood mononuclear cells (PBMCs) were analyzed. Real-time RT-PCR using the SYBR Green method was used to detect the expression level of these genes in PBMCs from 18 patients with AA and 14 healthy individuals. The β2microglobulin gene (β2M) was used as an endogenous reference. The expression levels of the CD3γ, CD3δ, CD3ε and CD3ζ genes in patients with AA were significantly increased compared to a healthy control group, whereas the FcεRIγ gene expression level was significantly decreased in patients with AA in comparison with the healthy control group. Moreover, the negative correlation of the expression levels between the CD3ζ and FcεRIγ genes was lost.
  
  
  CONCLUSIONS
  To our knowledge, this is the first report of the CD3γ, CD3δ, CD3ε, CD3ζ and FcεRIγ gene expression in patients with AA. The abnormally expressed TCR signaling related genes may relate to T cells dysfunction in AA.
authors.affiliation: Institute of Hematology, Medical College, Jinan University, Guangzhou 510632, China.
authors.forename: Bo
authors.initials: B
authors.lastname: Li
authors.forename: Sichu
authors.initials: S
authors.lastname: Liu
authors.forename: Yuzhe
authors.initials: Y
authors.lastname: Niu
authors.forename: Su
authors.initials: S
authors.lastname: Fang
authors.forename: Xiuli
authors.initials: X
authors.lastname: Wu
authors.forename: Zhi
authors.initials: Z
authors.lastname: Yu
authors.forename: Shaohua
authors.initials: S
authors.lastname: Chen
authors.forename: Lijian
authors.initials: L
authors.lastname: Yang
authors.forename: Yangqiu
authors.initials: Y
authors.lastname: Li
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000235:genetics*; D017252:CD3 Complex / Q000235:genetics*; D016022:Case-Control Studies; D002648:Child; D005260:Female; D005786:Gene Expression Regulation; D006801:Humans; D008297:Male; D008875:Middle Aged; D011948:Receptors, Antigen, T-Cell / Q000235:genetics / Q000378:metabolism; D017455:Receptors, IgE / Q000235:genetics*; D015398:Signal Transduction / Q000235:genetics / Q000276:immunology; D055815:Young Adult
chemical_list: D017252:CD3 Complex; C543372:FcepsilonRI gamma-chain, human; D011948:Receptors, Antigen, T-Cell; D017455:Receptors, IgE