Article

Recurrent infectious diseases in human CD53 deficiency.

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

ABSTRACT 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.

0 Bookmarks
 · 
79 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Toxoplasma gondii can establish chronic infection and is characterized by the formation of tissue cysts in the brain. Although T. gondii can infect any kind of nucleated cells, macrophages and related mononuclear phagocytes are its preferred targets in vivo. Microglial cells are the resident macrophages in the central nervous system. It has been reported that CD37, a tetraspanin molecule, is expressed exclusively in the immune system; Dectin-1, an important pattern-recognition receptor, is expressed on the surface of murine primary microglia. The Dectin-1-CD37 association can affect Dectin-1-mediated IL-6 secretion. However, there is no report concerning the relationship among the expressions of Dectin-1, IL-6, and CD37 during T. gondii infection. In the present study, Kunming outbred mice were infected with Prugniaud (Pru), a type II strain of T. gondii by oral gavage, and BV-2 murine microglial cells were cocultured with RH tachyzoites of T. gondii. By H&E and immunohistochemical staining, the results showed that marked inflammation and a significantly increased activation of Iba1-positive microglial cells were observed in the brain tissues of mice infected with T. gondii Pru strain at 5 weeks postinfection (p.i.) in comparison of uninfected controls. Using quantitative real-time PCR detection, Dectin-1 messenger RNA (mRNA) expressions were significantly upregulated in both brains at 3 (P < 0.01), 5 (P < 0.01), 7 (P < 0.01), and 9 (P < 0.05) weeks p.i. and spleens at 3, 5, 7, and 9 weeks p.i. (P < 0.01). IL-6 expressions showed similar dynamic tendency as that of Dectin-1 in both the brains and spleens at the same times in comparison of uninfected controls; CD37 expressions were significantly increased in the brain tissues at all the times (P < 0.01) and no significant differences in the spleens at 3 weeks p.i. but significantly downregulated in the spleens at 5, 7, and 9 weeks p.i. (P < 0.01). In vitro study showed that compared with uninfected controls, the mRNA expressions of Dectin-1 at 2, 4, 8, and 10 h (P < 0.01); IL-6 at 8 and 10 h (P < 0.01); and CD37 at 4 (P < 0.05), 8 (P < 0.01), and 10 h (P < 0.01) were significantly upregulated in BV-2 murine microglial cells stimulated with RH tachyzoites of T. gondii. Our data suggested that the expression of Dectin-1 was positively correlated with that of IL-6 in toxoplasmic encephalitis (TE) mouse model; Dectin-1 interaction with tetraspanin CD37 regulated IL-6 expression in both the brain tissues of TE mouse model and in the T. gongdii-infected BV-2 murine microglial cells.
    Parasitology Research 05/2014; · 2.33 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Gastrointestinal nematodes are one of the most serious causes of disease in domestic ruminants worldwide. There is considerable variation in resistance to gastrointestinal nematodes within and between sheep breeds, which appears to be due to underlying genetic diversity. Through selection of resistant animals, rapid genetic progress has been demonstrated in both research and commercial flocks. Recent advances in genome sequencing and genomic technologies provide new opportunities to understand the ovine host response to gastrointestinal nematodes at the molecular level, and to identify polymorphisms conferring nematode resistance.
    BMC Genomics 07/2014; 15(1):637. · 4.04 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The plasma membrane of immune cells is a highly organized cell structure that is key to the initiation and regulation of innate and adaptive immune responses. It is well-established that immunoreceptors embedded in the plasma membrane have a nonrandom spatial distribution that is important for coupling to components of intracellular signaling cascades. In the last two decades, specialized membrane microdomains, including lipid rafts and TEMs, have been identified. These domains are preformed structures ("physical entities") that compartmentalize proteins, lipids, and signaling molecules into multimolecular assemblies. In APCs, different microdomains containing immunoreceptors (MHC proteins, PRRs, integrins, among others) have been reported that are imperative for efficient pathogen recognition, the formation of the immunological synapse, and subsequent T cell activation. In addition, recent work has demonstrated that tetraspanin microdomains and lipid rafts are involved in BCR signaling and B cell activation. Research into the molecular mechanisms underlying membrane domain formation is fundamental to a comprehensive understanding of membrane-proximal signaling and APC function. This review will also discuss the advances in the microscopy field for the visualization of the plasma membrane, as well as the recent progress in targeting microdomains as novel, therapeutic approach for infectious and malignant diseases.
    Journal of leukocyte biology 10/2013; · 4.99 Impact Factor

Full-text (2 Sources)

Download
25 Downloads
Available from
Jun 1, 2014