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Cellular and Molecular Life Sciences CMLS

, Volume 61, Issue 18, pp 2361–2385 | Cite as

The Wiskott-Aldrich syndrome

  • J. S. OrangeEmail author
  • K. D. Stone
  • S. E. Turvey
  • K. Krzewski
Biomedicine and Diseases: Review

Abstract

The Wiskott-Aldrich Syndrome (WAS) is an inherited immunodeficiency caused by a variety of mutations in the gene encoding the WAS protein (WASp). WASp is expressed in hematopoetic cells and facilitates the reorganization of the actin cytoskeleton in response to many important cell stimuli. Extensive study of WAS and more recently WASp has given great insight into the relevance of this molecule and related molecules to both basic cell biology and human immune defenses.

Wiskott-Aldrich syndrome WASp cytoskeleton actin primary immunodeficiency 

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Copyright information

© Birkhäuser-Verlag Basel 2004

Authors and Affiliations

  • J. S. Orange
    • 1
    Email author
  • K. D. Stone
    • 2
  • S. E. Turvey
    • 3
  • K. Krzewski
    • 4
  1. 1.Division of Immunology, Children’s Hospital of Philadelphia, Department of PediatricsUniversity of Pennsylvania School of MedicinePhiladelphiaUSA
  2. 2.Division of Immunology, Children’s Hospital Boston, Department of PediatricsHarvard Medical SchoolBostonUSA
  3. 3.Division of Infections and Immunolocal Diseases, Department of PediatricsUniversity of British ColumbiaVacouverCanada
  4. 4.Department of Molecular and Cell BiologyHarvard UniversityCambridgeUSA

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