Cluster of Differentiation 40 (CD40L) Antibody – Mouse Monoclonal

CD40LG: 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. Defects in CD40LG are the cause of X-linked immunodeficiency with hyper-IgM type 1 (HIGM1); 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. Belongs to the tumor necrosis factor family.

Protein type: Cell adhesion; Membrane protein, integral

Chromosomal Location of Human Ortholog: Xq26.3

Cellular Component: cell surface; external side of plasma membrane; extracellular space; integral component of membrane; integral component of plasma membrane; intracellular; plasma membrane

Molecular Function: CD40 receptor binding; cytokine activity; protein binding; tumor necrosis factor receptor binding

Biological Process: activation of JNK activity; B cell differentiation; B cell proliferation; immunoglobulin secretion; inflammatory response; isotype switching; leukocyte adhesion; negative regulation of apoptosis; platelet activation; positive regulation of interleukin-10 production; positive regulation of interleukin-12 production; positive regulation of interleukin-4 production; positive regulation of NF-kappaB transcription factor activity; positive regulation of T cell proliferation; regulation of immune response; regulation of immunoglobulin secretion; T cell costimulation; tumor necrosis factor-mediated signaling pathway

Disease: Immunodeficiency With Hyper-igm, Type 1