نتایج جستجو برای: wiskott
تعداد نتایج: 1772 فیلتر نتایج به سال:
It is now recognized that CD3+ large granular lymphocyte (LGL) proliferations may be clonally derived from their normal CD3+LGL+ counterpart, but the nature of the pressure responsible for the proliferation of these cells remains unclear. We approached this problem by analyzing the diversity of the T-cell receptor repertoire of LGL developed in different clinical settings. Two of our patients h...
Signal transduction proteins that can integrate multiple upstream signals play a critical role in the complex regulatory circuits that control cellular behavior. The two signaling node proteins cyclin-dependent kinase 2 and the actin regulator neuronal Wiskott-Aldrich syndrome protein have qualitatively similar signaling properties. Recent studies, however, reveal that these proteins utilize di...
Chemotactic migration of macrophages is critical for the recruitment of leukocytes to inflamed tissues. Macrophages use a specialized adhesive structure called a podosome to migrate. Podosome formation requires the Wiskott-Aldrich syndrome protein (WASP), which is a product of the gene defective in an X-linked inherited immunodeficiency disorder, the Wiskott-Aldrich syndrome. Macrophages from W...
Src homology 3 (SH3) domains have been shown to mediate selected interactions between signaling molecules and are essential for the activation of a number of receptor-driven pathways. The Wiskott-Aldrich syndrome protein was identified as a protein that associated selectively with the SH3 domains derived from c-Src, p85alpha, phospholipase Cgamma1, and c-Fgr. Significantly reduced association w...
The actin nodule is a novel F-actin structure present in platelets during early spreading. However, only limited detail is known regarding nodule organization and function. Here we use electron microscopy, SIM and dSTORM super-resolution, and live-cell TIRF microscopy to characterize the structural organization and signalling pathways associated with nodule formation. Nodules are composed of up...
Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive primary immunodeficiency characterised by immune dysregulation, microthrombocytopaenia, eczema and lymphoid malignancies. Mutations in the WAS gene can lead to distinct syndrome variations which largely, although not exclusively, depend upon the mutation. Premature termination and deletions abrogate Wiskott-Aldrich syndrome protein (WA...
The high efficiency of Ag processing and presentation by B cells requires Ag-induced BCR signaling and actin cytoskeleton reorganization, although the underlying mechanism for such requirements remains elusive. In this study, we identify Bruton's tyrosine kinase (Btk) as a linker connecting BCR signaling to actin dynamics and the Ag transport pathway. Using xid mice and a Btk inhibitor, we show...
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