Recurrent infectious diseases in human CD53 deficiency. Clin Diagn Lab Immunol 4:229-231, PMCID: PMC170508

Instituto de Biología y Genética Molecular, Facultad de Medicina, CSIC-Universidad de Valladolid, Spain.
Clinical and Diagnostic Laboratory Immunology (Impact Factor: 2.51). 04/1997; 4(2):229-31.
Source: PubMed


We report a familiar syndrome of recurrent heterogeneous infectious diseases, caused by bacteria, fungi, and viruses, which has as its only detectable defect the lack of CD53 antigen expression in neutrophils. All other assays ruled out known causes of recurrent infectious diseases due to either leukocyte adhesion or phagocytosis defects. CD53 belongs to the transmembrane-4 superfamily of proteins, which are a novel group of membrane proteins implicated in growth regulation and cell motility and possibly cell adhesion. We postulate that defects in these membrane proteins can be clinically manifested as complex recurrent infections.

Download full-text


Available from: Pedro A. Lazo
  • Source
    • "Cd37−/− mice have a strikingly increased number of IgA+ plasma cells in their lymphoid organs, whereas the number of IgG+ plasma cells is low due to decreased survival signals in the GCs of the spleen (van Spriel et al. 2009, 2012). The function of tetraspanin CD53 has not been well studied although recurrent infections have been reported in a CD53-deficient family (Mollinedo et al. 1997). The underlying mechanism of tetraspanin function has been attributed to specific interactions between tetraspanins and immunoreceptors [major histocompatibility complex (MHC) proteins, B cell receptor (BCR), integrins and others] in the plasma membrane. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Multispectral imaging is a novel microscopy technique that combines imaging with spectroscopy to obtain both quantitative expression data and tissue distribution of different cellular markers. Tetraspanins CD37 and CD53 are four-transmembrane proteins involved in cellular and humoral immune responses. However, comprehensive immunohistochemical analyses of CD37 and CD53 in human lymphoid organs have not been performed so far. We investigated CD37 and CD53 protein expression on primary human immune cell subsets in blood and in primary and secondary lymphoid organs. Both tetraspanins were prominently expressed on antigen-presenting cells, with highest expression of CD37 on B lymphocytes. Analysis of subcellular distribution showed presence of both tetraspanins on the plasma membrane and on endosomes. In addition, CD53 was also present on lysosomes. Quantitative analysis of expression and localization of CD37 and CD53 on lymphocytes within lymphoid tissues by multispectral imaging revealed high expression of both tetraspanins on CD20+ cells in B cell follicles in human spleen and appendix. CD3+ T cells within splenic T cell zones expressed lower levels of CD37 and CD53 compared to T cells in the red pulp of human spleen. B cells in human bone marrow highly expressed CD37, whereas the expression of CD53 was low. In conclusion, we demonstrate differential expression of CD37 and CD53 on primary human immune cells, their subcellular localization and their quantitative distribution in human lymphoid organs. This study provides a solid basis for better insight into the function of tetraspanins in the human immune response. Electronic supplementary material The online version of this article (doi:10.1007/s00418-015-1326-2) contains supplementary material, which is available to authorized users.
    Full-text · Article · May 2015 · Histochemie
  • Source
    • "Based on the present and previous studies, the physiological function of CD53 appears to be to promote leukocyte adhesion and survival. Also, a study of humans with CD53-deficiency reported a disease phenotype indicating defects in cellular adhesion [51]. In addition to promoting adhesion on its own, CD53 ligation enhances LFA-1 activity induced by the activating receptor Ly49s3. "
    [Show abstract] [Hide abstract]
    ABSTRACT: NK cells express several tetraspanin proteins, which differentially modulate NK cell activities. The tetraspanin CD53 is expressed by all resting NK cells and was previously shown to decrease NK cell cytotoxicity upon ligation. Here, we show that CD53 ligation reduced degranulation of rat NK cells in response to tumour target cells, evoked redirected inhibition of killing of Fc-bearing targets, and reduced the IFN-γ response induced by plate-bound antibodies towards several activating NK cell receptors (Ly49s3, NKR-P1A, and NKp46). CD53 induced activation of the β2 integrin LFA-1, which was further enhanced upon co-stimulation with activating NK cell receptors. Concordant with a role for CD53 in increasing NK cell adhesiveness, CD53 ligation induced a strong homotypic adhesion between NK cells. Further, the proliferative capacity of NK cells to a suboptimal dose of IL-2 was enhanced by CD53 ligation. Taken together, these data suggest that CD53 may shift NK cell responses from effector functions towards a proliferation phase.
    Full-text · Article · May 2014 · PLoS ONE
  • Source
    • "These studies demonstrate that tetraspanin absence or dysfunction can result in the development of multiple phenotypic defects. Interestingly, human CD53 deficiency has been linked to recurrent infectious diseases caused by bacteria, fungi and viruses [11]. In the immune system, tetraspanins form functional interactions with prominent leukocyte receptors including MHC molecules, proteins of the B cell receptor complex, CD4, CD8, integrins and C-type lectins. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Tetraspanin proteins on host cells are involved in the pathogenesis of infectious diseases at different stages. In this review, we will focus on tetraspanins expressed in the immune system and the role they play in the defense to viral, bacterial, parasitic and fungal infections.
    Full-text · Article · Nov 2009 · Microbes and Infection
Show more