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Wiskott aldrich

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https://www.readbyqxmd.com/read/27911716/wasp-family-proteins-more-than-arp2-3-activators
#1
REVIEW
Joe J Tyler, Ellen G Allwood, Kathryn R Ayscough
Wiskott-Aldrich syndrome protein (WASP) family proteins have been extensively characterized as factors that promote the nucleation of actin through the activation of the protein complex Arp2/3. While yeast mostly have a single member of the family, mammalian cells have at least six different members, often with multiple isoforms. Members of the family are characterized by a common structure. Their N-termini are varied and are considered to confer spatial and temporal regulation of Arp2/3-activating activity, whereas their C-terminal half contains a polyproline-rich region, one or more WASP homology-2 (WH2) actin-binding domains and motifs that bind directly to Arp2/3...
October 15, 2016: Biochemical Society Transactions
https://www.readbyqxmd.com/read/27909303/conditional-knockout-of-n-wasp-in-mouse-fibroblast-caused-keratinocyte-hyper-proliferation-and-enhanced-wound-closure
#2
Neeraj Jain, Pazhanichamy Kalailingam, Kai Wei Tan, Hui Bing Tan, Ming Keat Sng, Jeremy Soon Kiat Chan, Nguan Soon Tan, Thirumaran Thanabalu
Neural-Wiskott Aldrich Syndrome Protein (N-WASP) is expressed ubiquitously, regulates actin polymerization and is essential during mouse development. We have previously shown that N-WASP is critical for cell-ECM adhesion in fibroblasts. To characterize the role of N-WASP in fibroblast for skin development, we generated a conditional knockout mouse model in which fibroblast N-WASP was ablated using the Cre recombinase driven by Fibroblast Specific Protein promoter (Fsp-Cre). N-WASP(FKO) (N-WASP(fl/fl); Fsp-cre) were born following Mendelian genetics, survived without any visible abnormalities for more than 1 year and were sexually reproductive, suggesting that expression of N-WASP in fibroblast is not critical for survival under laboratory conditions...
December 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27904039/role-of-rac1-wave2-signaling-in-mediating-the-inhibitory-effects-of-%C3%AE-tocotrienol-on-mammary-cancer-cell-migration-and-invasion
#3
Ibrahim Gayadh Algayadh, Venkateshwararao Dronamraju, Paul William Sylvester
The majority of breast cancer deaths result from the progression of this disease to a metastatic phenotype. Rac1 and Cdc42 are Rho family members that together with their downstream effectors, Wiskott-Aldrich Syndrome protein-family verprolin-homologous protein 2 (WAVE2) and Arp2/3, play an important role in cytoskeletal reorganization and the formation of membrane protrusions that promote cancer cell migration and invasion. γ-Tocotrienol, is a natural isoform within the vitamin E family of compounds that inhibits breast cancer cell growth and progression by suppressing various signaling pathways involved in mitogenic signaling and metastatic progression...
2016: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/27885891/wiskott-aldrich-syndrome-in-a-child-presenting-with-macrothrombocytopenia
#4
Jose Maria Bastida, Monica Del Rey, Nuria Revilla, Rocio Benito, Martin Perez-Andrés, Berta González, Susana Riesco, Kamila Janusz, Jose Padilla, Ana Hortal Benito-Sendin, David Bueno, Elena Blanco, Maria Hernández-Rivas, Vicente Vicente, Jose Rivera, Ramon González-Porras, Maria Luisa Lozano
Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive disease resulting from variants in the WAS gene, characterized by a triad of immunodeficiency, eczema, and thrombocytopenia. Despite the fact that WAS is traditionally differentiated from immune thrombocytopenia (ITP) by small size of WAS platelets, in practice, microthrombocytopenia may occasionally not be present, and in certain cases, WAS patients exhibit some parallelism to ITP patients. We characterized one patient presenting with the classic form of the disease but increased mean platelet volume...
November 25, 2016: Platelets
https://www.readbyqxmd.com/read/27884932/drosophila-wash-is-required-for-integrin-mediated-cell-adhesion-cell-motility-and-lysosomal-neutralization
#5
Benedikt M Nagel, Meike Bechtold, Luis Garcia Rodriguez, Sven Bogdan
The Wiskott-Aldrich Syndrome Protein and SCAR Homologue (WASH) is a conserved actin nucleation promoting factor controlling Arp2/3 complex activity in endosomal sorting and recycling. Previous studies have identified WASH as an essential regulator in Drosophila development. Here, we show that homozygous wash mutant flies are viable and fertile. We demonstrate that Drosophila WASH has conserved functions in integrin receptor recycling and lysosome neutralization. WASH generates actin patches on endosomes and lysosomes mediating both functions...
November 24, 2016: Journal of Cell Science
https://www.readbyqxmd.com/read/27829721/successful-engraftment-after-hematopoietic-stem-cell-transplantation-with-infusion-of-donor-stem-cells-through-the-extracorporeal-membrane-oxygenation-circuit
#6
Pilar Anton-Martin, Cindy Darnell-Bowens, Victor M Aquino, Teresa Jones, Lakshmi Raman
Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency due to mutations in the WAS gene expressed in hematopoietic cells. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice when an appropriate human leukocyte antigen-matched donor is available. The use of the extracorporeal membrane oxygenation (ECMO) circuit to infuse donor cells for HSCT has not been previously published in the literature. We describe a case of a child who had successful engraftment after HSCT with infusion of the donor stem cells through the ECMO circuit...
October 2016: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/27817076/retraction-note-to-clinical-significance-and-expression-of-the-prss3-and-wiskott-aldrich-syndrome-protein-family-verprolin-homologous-protein-1-for-the-early-detection-of-epithelial-ovarian-cancer
#7
Sima Azizmohammadi, Aghdas Safari, Mehri Seifoleslami, Rahman Ghaffarzadegan Rabati, Mohsen Mohammadi, Hamid Yahaghi, Susan Azizmohammadi
No abstract text is available yet for this article.
November 5, 2016: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/27778524/the-actin-binding-proteins-cortactin-and-hs1-are-dispensable-for-platelet-actin-nodule-and-megakaryocyte-podosome-formation
#8
Steven G Thomas, Natalie S Poulter, Danai Bem, Brenda Finney, Laura M Machesky, Stephen P Watson
A dynamic, properly organised actin cytoskeleton is critical for the production and haemostatic function of platelets. The Wiskott Aldrich Syndrome protein (WASp) and Actin-Related Proteins 2 & 3 Complex (Arp2/3 complex) are critical mediators of actin polymerisation and organisation in many cell types. In platelets and megakaryocytes, these proteins have been shown to be important for proper platelet production and function. The cortactin family of proteins (Cttn & HS1) are known to regulate WASp-Arp2/3-mediated actin polymerisation in other cell types and so here we address the role of these proteins in platelets using knockout mouse models...
October 25, 2016: Platelets
https://www.readbyqxmd.com/read/27760100/allergy-in-wiskott-aldrich-syndrome
#9
Zoltan Fehervari
No abstract text is available yet for this article.
October 19, 2016: Nature Immunology
https://www.readbyqxmd.com/read/27750026/gene-therapy-with-hematopoietic-stem-cells-the-diseased-bone-marrow-s-point-of-view
#10
Marina Cavazzana, Jean-Antoine Ribeil, Chantal Lagresle-Peyrou, Isabelle André-Schmutz
When considering inherited diseases that can be treated by gene transfer into hematopoietic stem cells (HSCs), there are only two in which the HSC and progenitor cell distribution inside the bone marrow and its microenvironment are exactly the same as in a healthy subject: metachromatic leukodystrophy (MLD) and adrenoleukodystrophy (ALD). In all other settings [X-linked severe combined immunodeficiency (X-SCID), adenosine deaminase deficiency, Wiskott-Aldrich syndrome, and β-hemoglobinopathies], the bone marrow content of the different stem and precursor cells and the cells' relationship with the stroma have very specific characteristics...
October 16, 2016: Stem Cells and Development
https://www.readbyqxmd.com/read/27742395/hematopoietic-stem-cell-transplantation-correctswip-deficiency
#11
Hamoud Al-Mousa, Abbas Hawwari, Abdulaziz Al-Ghonaium, Bandar Al-Saud, Hasan Al-Dhekri, Saleh Al-Muhsen, Sahar Elshorbagi, Majed Dasouki, Lina El-Baik, Amal Alseraihy, Mouhab Ayas, Rand Arnaout
WIP deficiency causes autosomal recessive Wiskott - Aldrich syndromewith normal platelet mean volume and can be cured with hematopoietic stem cell transplantation from genoidentical related or unrelated umbilical cord.
October 11, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27729544/the-c-terminal-dimerization-motif-of-cyclase-associated-protein-is-essential-for-actin-monomer-regulation
#12
Shohei Iwase, Shoichiro Ono
Cyclase-associated protein (CAP) is a conserved actin-regulatory protein that functions together with actin-depolymerizing factor (ADF)/cofilin to enhance actin filament dynamics. CAP has multiple functional domains, and the function to regulate actin monomers is carried out by its C-terminal half containing a Wiskott-Aldrich Syndrome protein homology 2 (WH2) domain, a CAP and X-linked retinitis pigmentosa 2 (CARP) domain, and a dimerization motif. WH2 and CARP are implicated in binding to actin monomers and important for enhancing filament turnover...
October 11, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27715449/cdc42-in-actin-dynamics-an-ordered-pathway-governed-by-complex-equilibria-and-directional-effector-handover
#13
Joanna R Watson, Darerca Owen, Helen R Mott
The small GTPase, Cdc42, is a key regulator of actin dynamics, functioning to connect multiple signals to actin polymerization through effector proteins of the Wiskott-Aldrich syndrome protein (WASP) and Transducer of Cdc42-dependent actin assembly (TOCA) families. WASP family members serve to couple Cdc42 with the actin nucleator, the Arp2/3 complex, via direct interactions. The regulation of these proteins in the context of actin dynamics has been extensively studied. Studies on the TOCA family, however, are more limited and relatively little is known about their roles and regulation...
August 11, 2016: Small GTPases
https://www.readbyqxmd.com/read/27703456/cancers-related-to-immunodeficiencies-update-and-perspectives
#14
Esmaeil Mortaz, Payam Tabarsi, Davod Mansouri, Adnan Khosravi, Johan Garssen, Aliakbar Velayati, Ian M Adcock
The life span of patients with primary and secondary immunodeficiency is increasing due to recent improvements in therapeutic strategies. While the incidence of primary immunodeficiencies (PIDs) is 1:10,000 births, that of secondary immunodeficiencies are more common and are associated with posttransplantation immune dysfunction, with immunosuppressive medication for human immunodeficiency virus or with human T-cell lymphotropic virus infection. After infection, malignancy is the most prevalent cause of death in both children and adults with (PIDs)...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27694894/npm-alk-phosphorylates-wasp-y102-and-contributes-to-oncogenesis-of-anaplastic-large-cell-lymphoma
#15
C A Murga-Zamalloa, V Mendoza-Reinoso, A A Sahasrabuddhe, D Rolland, S R Hwang, S R P McDonnell, A P Sciallis, R A Wilcox, V Bashur, K Elenitoba-Johnson, M S Lim
Mechanisms by which NPM-ALK signaling regulates cell migration, invasion and contributes to the oncogenesis of anaplastic large cell lymphoma (ALCL) are not completely understood. In an attempt to identify novel actin signaling pathways regulated by NPM-ALK, a comprehensive phosphoproteome analysis of ALCL cell lines was performed in the presence or absence of NPM-ALK activity. Numerous phosphoproteins involved in actin dynamics including Wiskott-Aldrich syndrome protein (WASp) were regulated by NPM-ALK. Network analysis revealed that WASp is a central component of the NPM-ALK-dependent actin signaling pathway...
October 3, 2016: Oncogene
https://www.readbyqxmd.com/read/27685868/the-cell-division-control-protein-42-src-family-kinase-neural-wiskott-aldrich-syndrome-protein-pathway-regulates-human-proplatelet-formation
#16
Alberta Palazzo, Olivier Bluteau, Kahia Messaoudi, Francesco Marangoni, Yunhua Chang, Sylvie Souquere, Gérard Pierron, Valérie Lapierre, Yi Zheng, William Vainchenker, Hana Raslova, Najet Debili
BACKGROUND: Cytoskeleton rearrangements are essential in platelet release. The RHO small GTPase family, as regulators of the actin cytoskeleton, plays an important function in proplatelet formation. In the neuronal system, CDC42 is involved in the axone formation, a process that combined elongation and branching as proplatelet formation. OBJECTIVE: To analyze the role of CDC42 and its effectors of the WASP family on proplatelet formation METHODS: Human MKs were obtained from CD34(+) cells...
September 29, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27668906/postnatal-cytomegalovirus-infection-in-an-infant-with-congenital-thrombocytopenia-how-it-can-support-or-mislead-the-diagnosis-of-wiskott-aldrich-syndrome
#17
Dimitri Poddighe, Elena Virginia, Marco Nedbal, Annarosa Soresina, Paola Bruni
A male newborn developed a post-natal cytomegalovirus (CMV) infection, arising in the clinical setting of congenital thrombocytopenia, which was diagnosed as being alloimmune. The evidence of active CMV infection in an infant showing slow-resolution lower airways infection, persistent neonatal and low platelet volume thrombocytopenia, and diffuse eczema (associated to very high levels of serum immunoglobulin E) led to the diagnosis of Wiskott-Aldrich syndrome (WAS) before the third month of life, despite the presence of several confounding clinical factors...
September 1, 2016: Le Infezioni in Medicina
https://www.readbyqxmd.com/read/27643438/foxp3-tregs-require-wasp-to-restrain-th2-mediated-food-allergy
#18
Willem S Lexmond, Jeremy A Goettel, Jonathan J Lyons, Justin Jacobse, Marion M Deken, Monica G Lawrence, Thomas H DiMaggio, Daniel Kotlarz, Elizabeth Garabedian, Paul Sackstein, Celeste C Nelson, Nina Jones, Kelly D Stone, Fabio Candotti, Edmond H H M Rings, Adrian J Thrasher, Joshua D Milner, Scott B Snapper, Edda Fiebiger
In addition to the infectious consequences of immunodeficiency, patients with Wiskott-Aldrich syndrome (WAS) often suffer from poorly understood exaggerated immune responses that result in autoimmunity and elevated levels of serum IgE. Here, we have shown that WAS patients and mice deficient in WAS protein (WASP) frequently develop IgE-mediated reactions to common food allergens. WASP-deficient animals displayed an adjuvant-free IgE-sensitization to chow antigens that was most pronounced for wheat and soy and occurred under specific pathogen-free as well as germ-free housing conditions...
October 3, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27643436/wasp-tregs-and-food-allergies-rare-disease-provides-insight-into-a-common-problem
#19
Yun Liang, Johann E Gudjonsson
Dysregulation of the type 2 immune system presents with various manifestations, including allergic inflammation, and has emerged as an alarming public health issue. The pathological mechanisms that underlie T helper type 2 cell-driven (Th2-driven) allergic diseases remain unclear. In particular, it is not completely understood how type 2 immunity is restricted in inflammatory responses. In this issue of the JCI, Lexmond et al. use Wiskott-Aldrich syndrome as a model disease and establish that the Wiskott-Aldrich gene product (WASP) serves an essential role in T regulatory cells to contain Th2 effector cell differentiation and prevent allergic sensitization to dietary antigens...
October 3, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27601635/coordinated-autoinhibition-of-f-bar-domain-membrane-binding-and-wasp-activation-by-nervous-wreck
#20
Tatiana B Stanishneva-Konovalova, Charlotte F Kelley, Tania L Eskin, Emily M Messelaar, Steven A Wasserman, Olga S Sokolova, Avital A Rodal
Membrane remodeling by Fes/Cip4 homology-Bin/Amphiphysin/Rvs167 (F-BAR) proteins is regulated by autoinhibitory interactions between their SRC homology 3 (SH3) and F-BAR domains. The structural basis of autoregulation, and whether it affects interactions of SH3 domains with other cellular ligands, remain unclear. Here we used single-particle electron microscopy to determine the structure of the F-BAR protein Nervous Wreck (Nwk) in both soluble and membrane-bound states. On membrane binding, Nwk SH3 domains do not completely dissociate from the F-BAR dimer, but instead shift from its concave surface to positions on either side of the dimer...
September 20, 2016: Proceedings of the National Academy of Sciences of the United States of America
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