Protein Connects Cell Signaling Systems with Cytoskeleton

The Wiskott-Aldrich syndrome (WAS) is a rare hereditary disease that causes immune system deficiencies and sometimes immune cell cancers. The disease is caused by a defective gene, and current research has revealed details of how the gene may cause WAS. In its normal form, the gene makes a protein, called WASp, that functions in connecting the cell's cytoskeleton with cell-signaling pathways in the plasma membrane. The WASp protein thereby allows the cell to receive information from its environment and make the appropriate changes in the cytoskeleton.

Scientists have suggested that, specifically, WASp may control the rearrangement of actin filaments in the cytoskeleton. The disassembling and reformation of actin filaments cause immune cells to move and are involved in the production of antibodies. Also, WASp may relay signals that tell immune cells to divide when many cells are needed to fight an infection. Thus, WASp is involved in how immune cells respond to infections and when immune cells reproduce. If the gene that codes for WASp is defective, the immune system will not function properly and the control of cell division is altered, which may lead to cancers.

"The Many Faces of WAS Protein," Science, vol. 275, page 27, January 3, 1997

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