Encyclopedia of Signaling Molecules

2018 Edition
| Editors: Sangdun Choi

N-WASP

  • Haein Park
  • Athanassios Dovas
  • Dianne Cox
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-67199-4_605

Synonyms

Historical Background

Wiskott–Aldrich syndrome (WAS), a pediatric disorder, was first described in 1937 by Alfred Wiskott as a “hereditary thrombopathy” in males, presenting with thrombocytopenia, eczema, bloody diarrhea, episodes of fever, and recurrent bacterial infections. Robert A. Aldrich would later demonstrate an X-linked mode of inheritance of this disease. Other features of WAS were later recognized, including immunodeficiency involving both humoral and cellular immunity, high rate of autoimmunity and malignancies, abnormal apoptosis, and defective cell motility. The mutated gene giving rise to this disease was identified in 1994 by positional cloning and referred to as WAS, and mutations of the WAS protein (WASP) were demonstrated not only in patients with WAS, but also in those with X-linked thrombocytopenia (XLT), a disease showing milder clinical phenotype with a more favorable...

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References

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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Anatomy and Structural BiologyAlbert Einstein College of Medicine, Jack and Pearl Resnick CampusBronxUSA