CD40 Ligand/CD40 Deficiency

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Functional defects of dendritic cells in patients with CD40 deficiency.

We have recently identified 2 patients with a rare autosomal recessive form of hyper IgM disease, known as HIGM3, caused by mutations in the CD40 gene. These patients had opportunistic infections observed on X-linked hyper IgM syndrome (HIGM), suggesting that the CD40-CD40 ligand interaction is important for promoting T-cell-mediated immunity. To evaluate whether innate immunity signals may sub...

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successful management of neutropenia in a patient with cd40 ligand deficiency by immunoglobulin replacement therapy

hyper-igm syndromes are characterized by profound reduction of serum igg, iga, and ige levels with normal or increased concentrations of serum igm. cd40 ligand deficiency is x-linked form of the disease, which results in a lack of immunoglobulin class switching from igm to igg in b cells. in addition to the recurrent infections, a number of patients suffer from neutropenia. there are some evide...

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Proangiogenic function of CD40 ligand-CD40 interactions.

Angiogenesis is a characteristic component of cell-mediated immune inflammation. However, little is known of the immunologic mediators of angiogenesis factor production. Interactions between CD40 ligand (CD40L) and CD40 have been shown to have pluripotent functions in inflammation, including the production of cytokines, chemokines, as well as the angiogenesis factor, vascular endothelial growth...

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Immune regulation by CD40-CD40-L interactions.

CD40 is a cell surface receptor, which belongs to the TNF-R family, and which was first identified and functionally characterized on B lymphocytes. However, in recent years it has become clear that CD40 is expressed much broader, including expression on monocytes, dendritic cells, endothelial cells and epithelial cells. Therefore it is now thought that CD40 plays a more general role in immune r...

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Bone marrow transplantation for CD40 ligand deficiency: a single centre experience.

BACKGROUND CD40 ligand (CD40L) deficiency is a rare X linked immunodeficiency disorder leading to recurrent bacterial infection, with cryptosporidial enteritis and subsequent hepatic cirrhosis. Bone marrow transplantation offers the only cure. OBJECTIVE To analyse retrospectively the outcome of bone marrow transplantation for this condition in one centre. DESIGN A retrospective case note an...

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ژورنال

عنوان ژورنال: International Archives of Allergy and Immunology

سال: 1995

ISSN: 1018-2438,1423-0097

DOI: 10.1159/000236923