Wiskott-Aldrich syndrome gene mutations modulate cancer susceptibility in the p53^± murine model.

ABSTRACT: The Wiskott-Aldrich syndrome protein (WASp) is a key regulator of the actin cytoskeleton in hematopoietic cells and mutated in two severe immunodeficiency diseases with high incidence of cancer. Wiskott-Aldrich syndrome (WAS) is caused by loss-of-function mutations in WASp and most frequently associated with lymphoreticular tumors of poor prognosis. X-linked neuropenia (XLN) is caused by gain-of-function mutations in WASp and associated with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). To understand the role of WASp in tumorigenesis, we bred WASp⁺, WASp^-, and WASp-XLN mice onto tumor susceptible p53^+/- background and sub-lethally irradiated them to enhance tumor development. We followed the cohorts for 24 weeks and tumors were characteri

SUBMITTER: Keszei M 

PROVIDER: S-EPMC6209425 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature