Abstract
The regulation of many immunological events depends on systems that mediate dynamic actin reorganization in response to signals from the cell membrane. The Wiskott-Aldrich syndrome protein (WASp) is the founding member of a family of proteins that have emerged as crucial effectors of Rho GTPases and activators of the cytoskeletal-organizing complex Arp2/3. Now, WASp has been shown to be intimately involved in many pathways that influence the function of the immune system. Disturbances in these systems result in the complex immunodysregulation of Wiskott-Aldrich syndrome.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Actin-Related Protein 2
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Actin-Related Protein 3
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Actins / metabolism
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Animals
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Blood Cells / physiology
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Cell Membrane / immunology
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Cytoskeletal Proteins / biosynthesis
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Humans
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Lymphocytes / immunology
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Microfilament Proteins / chemistry
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Microfilament Proteins / immunology
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Microfilament Proteins / physiology
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Nerve Tissue Proteins / deficiency
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Nerve Tissue Proteins / immunology
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Nerve Tissue Proteins / physiology
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Phagocytosis
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Proteins / chemistry
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Proteins / immunology
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Proteins / physiology*
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Signal Transduction
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Wiskott-Aldrich Syndrome / blood
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Wiskott-Aldrich Syndrome / immunology*
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Wiskott-Aldrich Syndrome / therapy
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Wiskott-Aldrich Syndrome Protein
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Wiskott-Aldrich Syndrome Protein Family
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Wiskott-Aldrich Syndrome Protein, Neuronal
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rho GTP-Binding Proteins / biosynthesis
Substances
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ACTR2 protein, human
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ACTR3 protein, human
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Actin-Related Protein 2
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Actin-Related Protein 3
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Actins
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Cytoskeletal Proteins
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Microfilament Proteins
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Nerve Tissue Proteins
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Proteins
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WAS protein, human
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WASL protein, human
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Wiskott-Aldrich Syndrome Protein
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Wiskott-Aldrich Syndrome Protein Family
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Wiskott-Aldrich Syndrome Protein, Neuronal
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rho GTP-Binding Proteins