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Rabbit Anti-CD40L/FITC Conjugated antibody
background:
CD40 ligand (CD40L) is a 33 kDa type II membrane glycoprotein expressed mainly on the cell surface of activated T lymphocytes, but also exists as a soluble form extracellularly. CD40L is the ligand for CD40, a member of the TNF superfamily, which is expressed on the cell surface of B cells, macrophages/monocytes, dendritic cells, vascular endothelial cells, and epithelial cells. CD40L plays an important role in B cell proliferation, antibody class switching, modulation of apoptosis in the germinal center through interaction with B cells expressing CD40, and activation of CD4+ T cells. Mutation within the CD40L gene is linked to hyper IgM syndrome, an X linked immunodeficiency disease that is characterized by elevated level of serum IgM and decreased level of other isotypes.
Function:
Mediates B-cell proliferation in the absence of co-stimulus as well as IgE production in the presence of IL-4. Involved in immunoglobulin class switching.
Release of soluble CD40L from platelets is partially regulated by GP IIb/IIIa, actin polymerization, and an matrix metalloproteinases (MMP) inhibitor-sensitive pathway.
Subcellular Location:
Secreted and Cell membrane.
Tissue Specificity:
Specifically expressed on activated CD4+ T-lymphocytes.
DISEASE:
Defects in CD40LG are the cause of X-linked immunodeficiency with hyper-IgM type 1 (HIGM1) [MIM:308230]; also known as X-linked hyper IgM syndrome (XHIM). HIGM1 is an immunoglobulin isotype switch defect characterized by elevated concentrations of serum IgM and decreased amounts of all other isotypes. Affected males present at an early age (usually within the first year of life) recurrent bacterial and opportunistic infections, including Pneumocystis carinii pneumonia and intractable diarrhea due to cryptosporidium infection. Despite substitution treatment with intravenous immunoglobulin, the overall prognosis is rather poor, with a death rate of about 10% before adolescence.
Similarity:
Belongs to the tumor necrosis factor family.
Database links:
Entrez Gene: 959 Human
Entrez Gene: 21947 Mouse
Entrez Gene: 84349 Rat
Omim: 300386 Human
SwissProt: P29965 Human
SwissProt: P27548 Mouse
SwissProt: Q9Z2V2 Rat
Unigene: 592244 Human
Unigene: 4861 Mouse
Unigene: 44218 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
CD40L也是T细胞活化的重要辅助刺激通路,不仅在调节T细胞免疫反应中起关键作用,而且也在B细胞的活化、增殖、分化、抗体的分泌起重要作用。主要表达在B细胞，有抗凋亡和促生长、分化的作用。
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