CD4+ cells from patients with Common Variable Immunodeficiency have a reduced ability of CD40 ligand membrane expression after in vitro stimulation

Background: Common Variable Immunodeficiency (CVID) is characterized by defective antibody production. This has been variably attributed to intrinsic B‐cell defects or to T‐cell disfunctions. Recently, it has been reported that the expression of the CD40 Ligand (CD40L), a T‐cell surface molecule that plays a critical role in the cell‐contact‐mediated helper signals provided to B‐cells, is defective in a subset of patients with CVID.

[1]  L. Notarangelo,et al.  Ineffective expression of CD40 ligand on cord blood T cells may contribute to poor immunoglobulin production in the newborn , 1994, European journal of immunology.

[2]  S. Meuer,et al.  Defective CD2 T cell pathway activation in common variable immunodeficiency (CVID) , 1994, Clinical and experimental immunology.

[3]  L. Notarangelo,et al.  Defective Expression of CD40 Ligand on T Cells Causes “X‐Linked Immunodeficiency with Hyper‐IgM (HIGM1)” , 1994, Immunological reviews.

[4]  J. Farrant,et al.  Failure in antigen responses by T ceils from patients with common variable immunodeficiency (CVID) , 1994, Clinical and experimental immunology.

[5]  R. Geha,et al.  Molecular Pathology of X‐Linked Immunoglobulin Deficiency with Normal or Elevated IgM (HIGMX‐1) , 1994, Immunological reviews.

[6]  H. Ochs,et al.  CD40 ligand expression is defective in a subset of patients with common variable immunodeficiency. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[7]  G. Inghirami,et al.  CD40 molecules induce down-modulation and endocytosis of T cell surface T cell-B cell activating molecule/CD40-L. Potential role in regulating helper effector function. , 1994, Journal of immunology.

[8]  H. Ochs,et al.  Activated B cells from patients with common variable immunodeficiency proliferate and synthesize immunoglobulin. , 1993, The Journal of clinical investigation.

[9]  D. Gray,et al.  Activated human T cells express a ligand for the human B cell‐associated antigen CD40 which participates in T cell‐dependent activation of B lymphocytes , 1992, European journal of immunology.

[10]  J. Farrant,et al.  Phenotypic and Functional Studies of Human Peripheral Blood Lymphocytes Engrafted in scid Mice , 1991, Immunological reviews.

[11]  J. Farrant,et al.  Defects in proliferative responses of T cells from patients with common variable immunodeficiency on direct activation of protein kinase C , 1991, Clinical and experimental immunology.

[12]  T. Waldmann,et al.  Characterization of common variable immunodeficiency: identification of a subset of patients with distinctive immunophenotypic and clinical features. , 1990, Blood.

[13]  P. Lipsky,et al.  T cell-dependent activation of B cell proliferation and differentiation by immobilized monoclonal antibodies to CD3. , 1988, Journal of immunology.

[14]  A. Rickinson,et al.  Functional 'immaturity' of isolated B cells from patients with hypogammaglobulinaemia. , 1980, Clinical and experimental immunology.

[15]  L. Notarangelo,et al.  Ontogeny of CD40 expression by activated peripheral blood lymphocytes in humans , 1996 .

[16]  R. Cattaneo,et al.  The ability of CD4+ cells from HIV+ individuals to express CD40 ligand after in vitro stimulation is not impaired. , 1995, Clinical immunology and immunopathology.

[17]  J. Banchereau,et al.  The CD40 antigen and its ligand. , 1994, Annual review of immunology.

[18]  J. Farrant,et al.  Study of B and T cell phenotypes in blood from patients with common variable immunodeficiency (CVID). , 1994, Immunodeficiency.

[19]  W. Strober,et al.  T-Cell Abnormalities in Common Variable Immunodeficiency , 1993, Pediatric Research.

[20]  J. Farrant,et al.  Cellular abnormalities in common variable immunodeficiency. , 1990, Immunodeficiency reviews.