Article
The structural basis of hyper IgM deficiency - CD40L mutations.
Protein engineering, design & selection : PEDS - 1 Mar 2007
Thusberg J, Vihinen M
Abstract excerpt
X-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency characterised by an inability to produce immunoglobulins of the IgG, IgA and IgE isotypes. It is caused by mutations of CD40 ligand (CD40L, CD154), expressed on T-lymphocytes. The interaction of CD40L on T-cells and its receptor CD40 on B-cells is essential for lymphocyte signalling leading to immunoglobulin class switching and B-cell maturation....
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