Existence of activated and memory CD4+ T cells in peripheral blood and their skin infiltration in CD8 deficiency

  title={Existence of activated and memory CD4+ T cells in peripheral blood and their skin infiltration in CD8 deficiency},
  author={Katamura and Tai and Tachibana and Yamabe and Ohmori and Mayumi and Matsuda and Koyasu and Furusho},
  journal={Clinical \& Experimental Immunology},
  • Katamura, Tai, Furusho
  • Published 1 January 1999
  • Biology, Medicine
  • Clinical & Experimental Immunology
CD8 deficiency is a rare primary immunodeficiency caused by the defect of a tyrosine kinase, ZAP‐70, which transduces signals from the T cell receptor. We report here a case of CD8 deficiency, having CD4+ T cells with a unique phenotype. The patient's T cells did not respond to anti‐CD3 stimulation in vitro, suggesting that they were naive. However, many CD4+ T cells with activated and memory phenotypes, which expressed CD45RO+, HLA‐DR+ and CD25+, were present in the peripheral blood, and these… 
Specific immunoglobulin E responses in ZAP‐70‐deficient patients are mediated by Syk‐dependent T‐cell receptor signalling
It was concluded that specific IgE responses in the patient were most likely to be mediated by Syk‐dependent TCR‐signalling.
Immune deficiencies , infection , and systemic immune disorders Characterization of z-associated protein , 70 kd ( ZAP 70 ) – deficient human lymphocytes
It is shown that in the absence of ZAP70, thymopoiesis is altered and differentiation to double-positive cells is hampered, a signature of autoimmunity.
Clinical heterogeneity can hamper the diagnosis of patients with ZAP70 deficiency
awareness of the clinical heterogeneity of ZAP70 deficiency is of utmost importance for making a fast and accurate diagnosis, which will contribute to the improvement of the adequate treatment of this severe immunodeficiency.
Hypomorphic mutation of ZAP70 in human results in a late onset immunodeficiency and no autoimmunity
This report reports an immunodeficiency with an inherited hypomorphic mutation of ZAP70 due to a single G‐to‐A substitution in a non‐coding intron that results in a compromised TCR signaling that was totally restored by increased expression of Zap70, demonstrating that defective activation of the patient T cells was indeed caused by the low level of Z AP70 expression.
Primary T-cell immunodeficiency with immunodysregulation caused by autosomal recessive LCK deficiency.
Impaired T cell receptor signaling and development of T cell–mediated autoimmune arthritis
These findings obtained from the study of SKG mouse arthritis are instrumental in understanding how arthritogenic T cells are produced, become activated, and differentiate into effector T cells mediating arthritis, and may help devising therapeutic measures targeting autoimmune pathogenic Th17 cells or autoimmune‐suppressing Treg cells to treat and prevent RA.
Combined Immune Deficiencies


Depletion of CD8+ cells in human thymic medulla results in selective immune deficiency
Data are consistent with an impaired selection of CD8+ cells in the patient's thymus and support the role of the CD8 surface protein in thymic selection previously characterized in genetically manipulated and inbred mice.
Absence of ZAP-70 prevents signaling through the antigen receptor on peripheral blood T cells but not on thymocytes
It is shown that the TCRs on thymocytes, but not peripheral T cells, from a ZAP-70-deficient patient are capable of signaling, and the homologous kinase Syk is present at high levels and is tyrosine phosphorylated after TCR stimulation.
Differential T cell receptor‐mediated signaling in naive and memory CD4 T cells
Investigation of proximal signaling events triggered through the TCR/CD3 complex in naive and memory CD4 T cell subsets isolated on the basis of CD45 isoform expression indicates that proximal signals are differentially coupled to the T CR in naive versus memoryCD4 T cells, potentially leading to distinct downstream signaling events and ultimately to the diverse functions elicited by these two CD 4 Tcell subsets.
Crucial Role of Jak3 in Negative Selection of Self-reactive T Cells
A crucial function of Jak3 is demonstrated in the negative selection of autoreactive T cells and the maintenance of functional peripheral T cells in Jak3-deficient mice.
A hereditary immunodeficiency characterized by CD8 T lymphocyte deficiency and impaired lymphocyte activation
The combination of an activation defect and selective depletion of CD8+ T lymphocytes suggests that the defective pathway is important in the differentiation of immature thymocytes as well as the proliferation of mature lymphocytes.
Essential role for ZAP-70 in both positive and negative selection of thymocytes
The data suggest that ZAP-70 is a central signalling molecule during thymic selection for CD4 and CD8 lineage, and that natural killer cell function was intact in the absence of Zap-70.
Peripheral Expression of Jak3 Is Required to Maintain T Lymphocyte Function
Jak3 expression in the thymus restores normal T cell development, including CD8+, γδ, and natural killer cells, however, the loss of Jak3 protein in peripheral T cells leads to the Jak3 −/− phenotype, demonstrating that Jak3 is constitutively required to maintain T cell function.
Variant type of sialyl Lewis X antigen expressed on adult T cell leukemia cells is associated with skin involvement.
Findings suggest that the expression of 2F3-defined sialyl Le(x) antigen on ATL cells is associated with skin involvement of ATL, which is an ATL cell-rich population.
Identification of circulating maternal T and B lymphocytes in uncomplicated severe combined immunodeficiency by HLA typing of subpopulations of T cells separated by the fluorescence-activated cell sorter and of Epstein Barr virus-derived B cell lines.
The presence of maternal T and B cells in uncomplicated SCID may be more common than thought previously and calls for a careful assessment of the origin of any mature T cells that are present in affected infants.