Wiskott Aldrich syndrome

Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive disease. Affected individuals suffer from immune dysregulation and microthrombocytopenia. They demonstrate a susceptibility to pyogenic, viral and opportunistic infection, and eczema. Immunologically, WAS is characterised by a progressive loss of T cells resulting in abnormal cell-mediated and DTH responses and abnormal antibody responses.

The genetic basis of the disease has been unravelled by the identification of a disease-specific protein - WASP (Wiskott-Aldrich syndrome protein). The WASP gene encodes a protein found solely in cells of haemopoietic origin. The WAS protein is involved in transporting signals from cell surface receptors to the cytoskeleton. Abnormalities in WASP lead to defects in the organisation of the cytoskeleton such that cellular motility (and therefore trafficking) is adversely affected.

5.3.5 T and B cell abnormalities

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