CD40LG

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CD40LG

A gene on chromosome Xq26 that encodes of a protein expressed on the surface of T cells which regulates B-cell function by binding CD40 to the B-cell surface. CD40LG is critical for immunoglobulin class switching. 

Molecular pathology
A defect in CD40LG is associated with hyper IgM syndrome due to an inability to undergo class switch.
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HSCT owing to its better outcomes is now increasingly practiced for treating non-SCID based patients suffering from diseases such as Wiskott-Aldrich syndrome, Omenn syndrome, CD40 ligand deficiency and others.
His cousins Paul and Glen also died from CD40 ligand deficiency and the family was told Patrick's only hope was a bone marrow transplant.
At birth Patrick's parents were told he was CD40 ligand deficient and the only way he would survive would be to have a bone marrow transplant.
Human bone marrow megakaryocytes and platelets express PPAR-[gamma], and PPAR-[gamma] agonists blunt platelet release of CD40 ligand and thromboxanes.
This molecule participates in initiation and maintenance of inflammation through interaction with CD40 ligand (CD40L or CD154) [5].
CD40 ligand (CD40L/CD154), a transmembrane glycoprotein retaining structural homology with tumor necrosis factor-[alpha] (TNF-[alpha]), was initially revealed on activated T lymphocytes (9).
The biomarkers measured included those associated with systemic inflammation (fibrinogen, C-reactive protein [CRP], white blood cell [WBC] count) and thrombosis or endothelial dysfunction (platelet activation markers P-selectin [sCD62P] and soluble CD40 ligand [sCD40L] as well as the adhesive endothelial glycoprotein von Willebrand factor).
noted a significant increase in CD40 ligand expression on monocytes from baseline during hypoglycemia in individuals with type 1 diabetes, although a significant confounding decrease in plasma CD40 ligand then occurred in the same group during recovery and at 6 hours.
RESULTS: Compared with air, particle exposure increased platelet expression of CD40 ligand (CD40L) and the number of platelet-leukocyte conjugates 3.
Soluble CD40 ligand (sCD40L) is expressed on platelets and released from them on activation.
CD40 ligand (CD40L) is expressed on T-cell and specifically binds to CD40.