Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038362 (stomatitis)
 8,852 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Job' syndrome and IgA immunodeficiency are a rare dysfunction of the immune system. In this work, we reported a case of a young woman who had recurrent episodes of bacterial infections in the urinary tract and genital, generalized erythematous eczematous patches and stomatitis of oral mucosa and fever. During the hospitalization, laboratory data showed high immunoglobulin IgE and low IgA levels. The T-lymphocyte presented a reduction of CD8+ cells. Tests of granulocyte function have showed a global deficit in the in vitro and in vivo chemotaxis. The correlation between these two clinic conditions is not completely clarified but it is possible to hypothesize that CD8+ lymphocytes produce an inhibition factor of chemotaxis. Job' syndrome is characterized by a selective reduction of CD8+ cells subpopulation which have an immunoregulatory function on the production of IgE by plasmacells. In the ipoIgA, an intrinsic inability of B-IgA cells to proliferate and to differentiate produce a defect in the IgA production. In these two clinic disorders there is an effective dysfunction of immune system. It is possible to hypothesize that an effective defect of CD8+ cells and an immaturity of B-cells may coexist in our patient. That justifies an abnormal production of Ig and a defect in granulocyte chemotaxis.
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PMID:[Job syndrome (hyper-IgE) and hypo-IgA. A rare association of immunodeficiencies]. 872 83

Dendritic cells (DC) are key cells of the innate immune system required to prime adaptive immunity. Central DC functions including antigen uptake and presentation and DC migration are critically dependent on dynamic cytoskeletal reorganisation, the regulation of which remains poorly understood. Cytoskeletal studies are complicated by the fact that DC cytoarchitecture is altered considerably by maturation stimuli, including many tools employed for biological manipulation. Lentiviral vectors, capable of transducing non-dividing cells such as DC, hold promise both for experimental and therapeutic manipulation of DC gene and protein expression but controversy remains about their effect on DC maturation. Here, we have examined the potential of lentiviral vectors as tools for gene delivery to monocyte derived human DC with preservation of immature DC cytoskeletal structure and function. We show that vesicular stomatitis virus G glycoprotein (VSVG)-pseudotyped lentivectors are most efficient at transducing immature DC and their precursor monocytes. Even at high multiplicities of infection transduced DC retained an immature cytoskeletal phenotype, with no significant alteration of migration, antigen uptake or T-cell stimulation capacities. Furthermore, lentivectors did not alter subsequent functional maturation of the DC cytoskeleton in response to lipopolysaccharide exposure. Together our data show that VSVG-psudotyped lentiviral vectors are an effective tool for gene manipulation in human DC with preservation of functional immaturity and plasticity, making them ideal for studies of the DC cytoskeleton.
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PMID:Lentivectors are efficient tools to manipulate the dendritic cell cytoskeleton. 2175 10