Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
We discover a key biochemical difference between Dip1 and WASP that may limit linear filament nucleation in cells; although WASP must be released for nucleation, Dip1 stays associated with Arp2/3 complex on the pointed ends of nucleated actin filaments, so Dip1 is consumed in the reaction.
|
31564494 |
2019 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
The discovery of Sca2 as an actin nucleator followed the identification of what appeared to be a repeat of three Wiskott-Aldrich syndrome homology 2 (WH2) domains in the middle of the molecule, consistent with the presence of WH2 domains in most actin nucleators.
|
30638962 |
2019 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
WASH, a Wiskott-Aldrich syndrome (WAS) family protein, has many cell and developmental roles related to its function as a branched actin nucleation factor.
|
29549166 |
2018 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
TrkC activation led to a mobility shift of Wiskott-Aldrich syndrome family verprolin-homologous protein (WAVE)-2 which is known to orchestrate Arp2/3 activation and actin polymerization.
|
29162704 |
2018 |
Wiskott-Aldrich Syndrome
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
We found that the subcellular location and the levels of of F-actin were altered in T cells from both WAS and XLT patients compared to that of HCs with or without stimulation.
|
28931895 |
2017 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
We propose actin nodules are platelet podosome-related structures required for platelet-platelet interaction and their absence contributes to the bleeding diathesis of Wiskott-Aldrich syndrome.
|
26028144 |
2015 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
Mutations in the gene encoding the Wiskott-Aldrich syndrome protein (WASP) are responsible for Wiskott-Aldrich syndrome and WASP is a major actin regulator in the cytoplasm.
|
24402308 |
2014 |
Wiskott-Aldrich Syndrome
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Wiskott-Aldrich syndrome (WAS) and X-linked neutropenia (XLN) are immunodeficiencies in which the function of several haematopoietic cell lineages is perturbed as a result of mutations in the actin regulator WASp.
|
23868979 |
2013 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
Together, these data highlight the role of actin dynamics in pDC innate functions and imply the pDC-IFN-α axis as a player in the onset of autoimmune phenomena in WAS disease.
|
23337808 |
2013 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Wiskott-Aldrich syndrome (WAS) protein (WASP) family is critical for productive T-cell receptor signaling and actin reorganization.
|
22674044 |
2012 |
Wiskott-Aldrich Syndrome
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
A peptide derived from the Wiskott-Aldrich syndrome (WAS) protein-interacting protein (WIP) restores WAS protein level and actin cytoskeleton reorganization in lymphocytes from patients with WAS mutations that disrupt WIP binding.
|
21376381 |
2011 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
Wiskott-Aldrich syndrome verprolin-homologous 3 (WAVE3) belongs to Wiskott-Aldrich syndrome family proteins (WASP), which, along with other members, play a critical role in the regulation of actin polymerization and cell motility.
|
19395286 |
2010 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Wiskott-Aldrich syndrome: The actin cytoskeleton and immune cell function.
|
21178275 |
2010 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Wiskott-Aldrich syndrome (WAS) protein (WASp) is a regulator of actin cytoskeleton in hematopoietic cells.Mutations of the WASp gene cause WAS.
|
19307326 |
2009 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
WASP-dependent F-actin polarization to the site of TCR triggering might not be involved in WAS(-/-) nTreg cell defects because this process was also inefficient in wild-type (WT) nTreg cells.
|
17296785 |
2007 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
We have used both the acidic domains from actin-related protein (Arp) 2/3 complex-binding proteins such as the Wiscott-Aldrich syndrome protein (N-WASP) or cortactin, and siRNA directing toward Arp2 to inhibit viral infection.
|
15385624 |
2004 |
Wiskott-Aldrich Syndrome
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Furthermore, the C-terminal CA domain of N-WASp, which sequesters Arp2/3 complex, inhibits by half the actin nucleation capacity of octylglucoside-permeabilized and activated WAS platelets, similar to its effect in WASp-expressing cells.
|
12200375 |
2002 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
The deficient chemotactic responses of WAS macrophages following cytokine stimulation could be correlated with abnormalities in cell polarisation and actin organisation.
|
11950596 |
2002 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
Furthermore, after CD3 cross-linking, transduced WAS T lines showed improvement of actin polymerization and T-cell receptor/CD3 down-regulation.
|
12067437 |
2002 |
Wiskott-Aldrich Syndrome
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency disorder caused by a mutation in WAS protein (WASp) that results in defective actin polymerization.
|
12177428 |
2002 |
Wiskott-Aldrich Syndrome
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
This suggests that the accelerated apoptosis observed in WAS lymphocytes was not secondary to an underlying defect in actin polymerization caused by mutation of the WAS gene.
|
10666201 |
2000 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
The Wiskott-Aldrich syndrome: disordered actin dynamics in haematopoietic cells.
|
11213796 |
2000 |
Wiskott-Aldrich Syndrome
|
0.100 |
Biomarker
|
disease |
BEFREE |
However, despite this abnormal filopodia formation, MKs from WAS patients normally migrated in response to stroma-derived factor-1alpha (SDF-1alpha), and actin normally polymerized after SDF-1alpha or thrombin stimulation.
|
10397718 |
1999 |
Wiskott-Aldrich Syndrome
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In addition, retrovirus-mediated expression of WASP led to improvement of cytoplasmic F-actin expression and formation of F-actin-positive microvilli, a process shown to be defective in untransduced WAS cell lines.
|
10455421 |
1999 |
Wiskott-Aldrich Syndrome
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The Wiskott-Aldrich syndrome (WAS) is a human X-linked immunodeficiency resulting from mutations in a gene (WASP) encoding a cytoplasmic protein implicated in regulating the actin cytoskeleton.
|
9697838 |
1998 |