A peptide derived from the Wiskott-Aldrich syndrome (WAS) protein-interacting protein (WIP) restores WAS protein level and actin cytoskeleton reorganization in …
MJ Massaad, N Ramesh, S Le Bras, S Giliani… - Journal of allergy and …, 2011 - Elsevier
BACKGROUND: The Wiskott-Aldrich syndrome (WAS) and X-linked thrombocytopenia (XLT)
are caused by mutations in WAS, which encodes for WAS protein (WASP). The WASP-
interacting protein (WIP) stabilizes WASP, as evidenced by severely decreased WASP
levels in T cells from WIP-deficient mice. The majority of missense mutations in patients with
WAS/XLT are located in the WIP-binding domain of WASP and might result in dissociation of
the WASP-WIP complex and WASP degradation. OBJECTIVE: To restore WASP levels and …
are caused by mutations in WAS, which encodes for WAS protein (WASP). The WASP-
interacting protein (WIP) stabilizes WASP, as evidenced by severely decreased WASP
levels in T cells from WIP-deficient mice. The majority of missense mutations in patients with
WAS/XLT are located in the WIP-binding domain of WASP and might result in dissociation of
the WASP-WIP complex and WASP degradation. OBJECTIVE: To restore WASP levels and …