CD154

CD154NCBI

CD154

Mouse myeloma cell line transfected with human CD40L (CD154)

Purified IgG prepared by affinity chromatography on Protein A

Flow Cytometry - 1/10 - 1/50

Release of soluble CD40L from platelets is partially regulated by GP IIb/IIIa actin polymerization and an matrix metalloproteinases (MMP) inhibitor-sensitive pathway.

Cell membrane; Single-pass type II membrane protein.

Specifically expressed on activated CD4+ T-lymphocytes.

N-linked glycan is a mixture of high mannose and complex type. Glycan structure does not influence binding affinity to CD40.

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.