Search Results

• • 38 

    Academic Journals

  • Sort by: sorttype.publication.date.desc Newest sorttype.publication.date.asc Oldest sorttype.relevance Relevance

• c-Src and Neural Wiskott-Aldrich Syndrome Protein (N-WASP) Promote Low Oxygen-Induced Accelerated Brain Invasion by Gliomas

  Authors:Zhuo Tang, Lita M. Araysi, and Hassan M Fathallah-ShaykhFrom:PLoS ONE (Vol. 8, Issue 9) Peer-Reviewed

  Sept. 19, 20137,025 words Article 1520LAuthor(s): Zhuo Tang 1, Lita M. Araysi 1, Hassan M Fathallah-Shaykh 1,2,3,4,5,6,7,* Introduction Motility is not only critically relevant to the understanding and therapeutics of cancer but is also important in...

• Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report

  Authors:Kostas N. Syrigos, Nektaria Makrilia, Jeffrey Neidhart, Michael Moutsos, Sotirios Tsimpoukis, Maria Kiagia, et al.From:Italian Journal of Pediatrics (Vol. 37) Peer-Reviewed

  Sept. 10, 20112,567 words Case study 1390LWiskott-Aldrich syndrome is a rare X-linked immunodeficiency disorder that is characterized by a variable clinical phenotype. Matched donor bone marrow transplantation is currently the only curative therapeutic option....

• The Chemotactic Defect in Wiskott-Aldrich Syndrome Macrophages Is Due to the Reduced Persistence of Directional Protrusions

  Authors:Dan Ishihara, Athanassios Dovas, Haein Park, Beth M. Isaac, and Dianne CoxFrom:PLoS ONE (Vol. 7, Issue 1) Peer-Reviewed

  Jan. 18, 20126,932 words Article 1600LWiskott-Aldrich syndrome protein (WASp) is an actin nucleation promoting factor that is required for macrophages to directionally migrate towards various chemoattractants. The chemotaxis defect of WASp-deficient cells...

• The many faces of the WAS protein

  Author:Carol FeatherstoneFrom:Science (Vol. 275, Issue 5296) Peer-Reviewed

  Jan. 3, 19972,088 words Article Recent research has revealed that WASp, the protein coded by the gene responsible for Wiskott-Aldrich syndrome, is a versatile molecule that connects the cytoskeleton and cellular signal transduction pathways....

• Phase transitions in the assembly of multivalent signalling proteins

  Authors:Pilong Li, Sudeep Banjade, Hui-Chun Cheng, Soyeon Kim, Baoyu Chen, Liang Guo, et al.From:Nature (Vol. 483, Issue 7389) Peer-Reviewed

  Mar. 15, 20125,160 words Report 1460LCells are organized on length scales ranging from angstrom to micrometres. However, the mechanisms by which angstrom-scale molecular properties are translated to micrometre-scale macroscopic properties are not well...

• Lentiviral vectors targeting WASp expression to hematopoietic cells, efficiently transduce and correct cells from WAS patients

  Authors:S Charrier, L Dupre, S Scaramuzza, L Jeanson-Leh, M P Blundell, O Danos, et al.From:Gene Therapy (Vol. 14, Issue 5) Peer-Reviewed

  Mar. 20079,440 words Article 1830LGene therapy has been proposed as a potential treatment for Wiskott-Aldrich syndrome (WAS), a severe primary immune deficiency characterized by multiple hematopoietic-specific cellular defects. In order to develop an...

• Wiskott-Aldrich syndrome with possible congenital Cytomegalovirus infection: A diagnostic dilemma.

  Authors:Behnam Sobouti, Ahmad Bahrami, Farzaneh Rahmani, Saeed Talebi, Vida Sherafati, Maryam Vafapour, et al.From:The National Medical Journal of India (Vol. 34, Issue 1) Peer-Reviewed

  Jan-Feb 20212,285 words Article 2030LByline: Behnam. Sobouti, Ahmad. Bahrami, Farzaneh. Rahmani, Saeed. Talebi, Vida. Sherafati, Maryam. Vafapour, Nima. Rezaei Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder, characterized by...

• Autoimmunity in Wiskott-Aldrich syndrome: risk factors, clinical features, and outcome in a single-center cohort of 55 patients

  Authors:Sophie Dupuis-Girod, Jacques Medioni, Elie Haddad, Pierre Quartier, Marina Cavazzana-Calvo, Francoise Le Deist, et al.From:Pediatrics (Vol. 111, Issue 5) Peer-Reviewed

  May 2003267 words Author abstract Objectives. To evaluate the occurrence of autoimmune and inflammatory complications in Wiskott-Aldrich syndrome (WAS) and to determine risk factors and the prognosis of such complications with the aim of improving the...

• Current understanding of the Wiskott-Aldrich syndrome and prospects for gene therapy

  Authors:Sara Trifari, Francesco Marangoni, Samantha Scaramuzza, Alessandro Aiuti, Maria Grazia Roncarolo, and Loic DupreFrom:Expert Review of Clinical Immunology (Vol. 3, Issue 2) Peer-Reviewed

  Mar. 20078,686 words Article Author(s): Sara Trifari 1 , Francesco Marangoni 2 , Samantha Scaramuzza 3 , Alessandro Aiuti 4 , Maria Grazia Roncarolo [[dagger]] 5 , Loïc Dupré 6 Keywords: gene therapy; hematopoietic stem cells; lentiviral...

• The Wiskott-Aldrich syndrome: from genotype-phenotype correlation to treatment

  Authors:Daniele Moratto, Silvia Giliani, Lucia D Notarangelo, Cinzia Mazza, Evelina Mazzolari, and Luigi D NotarangeloFrom:Expert Review of Clinical Immunology (Vol. 3, Issue 5) Peer-Reviewed

  Sept. 20079,708 words Article Author(s): Daniele Moratto 1 , Silvia Giliani 2 , Lucia D Notarangelo 3 , Cinzia Mazza 4 , Evelina Mazzolari 5 , Luigi D Notarangelo [[dagger]] 6 Keywords: clinical treatment; mutation analysis; myelodisplasia;...

• A DOCK8-WIP-WASp complex links T cell receptors to the actin cytoskeleton

  Authors:Erin Janssen, Mira Tohme, Mona Hedayat, Marion Leick, Sudha Kumari, Narayanaswamy Ramesh, et al.From:Journal of Clinical Investigation (Vol. 126, Issue 10) Peer-Reviewed

  Oct. 20168,977 words Report 1430LWiskott-Aldrich syndrome (WAS) is associated with mutations in the WAS protein (WASp), which plays a critical role in the initiation of T cell receptor-driven (TCR-driven) actin polymerization. The clinical phenotype of...

• Cidofovir: first report of oedema: case report

  From:Reactions Weekly (Issue 1236)

  Jan. 24, 2009127 words Brief article, Case ... [*] A 43-year-old man with a history of Wiskott-Aldrich syndrome had developed molluscum contagiosum, which had gradually involved his trunk, and lower and upper extremities over 1.5 years. This was treated with...

• N-WASP helps hair growth

  From:Journal of Clinical Investigation (Vol. 120, Issue 2) Peer-Reviewed

  Feb. 2010173 words Abstract, Brief arti... 1600LMany physiological processes in the skin, including wound healing and hair follicle (HF) cycling, involve actin cytoskeleton reorganization regulated by the Rho family GTPases Cdc42 and Rac1. Consistent with this,...

• Src kinase Hck association with the WASp and mDia1 cytoskeletal regulators promotes chemoattractant-induced Hck membrane targeting and activation in neutrophils

  Authors:Yongquan Shi, Baoxia Dong, Helen Miliotis, Junye Liu, Arthur S. Alberts, Jinyi Zhang, et al.From:Biochemistry and Cell Biology (Vol. 87, Issue 1) Peer-Reviewed

  Feb. 20096,222 words Report The haemopoietic cell kinase (Hck) plays an important but poorly understood role in coupling chemoattractant stimuli to the actin cytoskeletal rearrangement required for neutrophil polarization and chemotaxis. Here, we...

• Isolated EBV lymphoproliferative disease in a child with Wiskott-Aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy. (Case Report)

  Authors:N.J. Sebire, S. Haselden, M. Malone, E.G. Davies, and A.D. RamsayFrom:Journal of Clinical Pathology (Vol. 56, Issue 7) Peer-Reviewed

  July 20031,712 words Article Patients with primary immunodeficiencies such as the Wiskott-Aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients...

• Wiskott-Aldrich syndrome protein is required for regulatory T cell homeostasis

  Authors:Stephanie Humblet-Baron, Blythe Sather, Stephanie Anover, Shirly Becker-Herman, Debora J. Kasprowicz, Socheath Khim, et al.From:Journal of Clinical Investigation (Vol. 117, Issue 2) Peer-Reviewed

  Feb. 20077,629 words Article 1590LWiskott-Aldrich syndrome protein (WASp) is essential for optimal T cell activation. Patients with WAS exhibit both immunodeficiency and a marked susceptibility to systemic autoimmunity. We investigated whether...

• Validation of a mutated PRE sequence allowing high and sustained transgene expression while abrogating WHV-X protein synthesis: application to the gene therapy of WAS

  Authors:M.A. Zanta-Boussif, S. Charrier, A. Brice-Ouzet, S. Martin, P. Opolon, A.J. Thrasher, et al.From:Gene Therapy (Vol. 16, Issue 5) Peer-Reviewed

  May 20098,496 words Report The woodchuck hepatitis virus posttranscriptional regulatory element (WPRE) is widely used in retroviral gene transfer vectors. However, this element contains an open-reading frame (ORF) encoding a truncated peptide of...

• Neural Wiskott-Aldrich syndrome protein modulates Wnt signaling and is required for hair follicle cycling in mice

  Authors:Anna Lyubimova, John J. Garber, Geeta Upadhyay, Andrey Sharov, Florentina Anastasoaie, Vijay Yajnik, et al.From:Journal of Clinical Investigation (Vol. 120, Issue 2) Peer-Reviewed

  Feb. 20107,762 words Report 1430LThe Rho family GTPases Cdc42 and Rac1 are critical regulators of the actin cytoskeleton and are essential for skin and hair function. Wiskott-Aldrich syndrome family proteins act downstream of these GTPases, controlling...

• IL-2 induces a WAVE2-dependent pathway for actin reorganization that enables WASP-independent human NK cell function

  Authors:Jordan S. Orange, Sumita Roy-Ghanta, Emily M. Mace, Saumya Maru, Gregory D. Rak, Keri B. Sanborn, et al.From:Journal of Clinical Investigation (Vol. 121, Issue 4) Peer-Reviewed

  Apr. 20118,520 words Report 1440LWiskott-Aldrich syndrome (WAS) is a primary immunodeficiency associated with an increased susceptibility to herpesvirus infection and hematologic malignancy as well as a deficiency of NK cell function. It is caused by...

• The ups and downs of negative (and positive) selection of B cells

  Authors:Jean-Claude Weill and Claude-Agnes ReynaudFrom:Journal of Clinical Investigation (Vol. 125, Issue 10) Peer-Reviewed

  Oct. 20151,821 words Viewpoint essay 1570LCentral and peripheral tolerance checkpoints are In place to remove autoreactlve B cell populations and prevent the development of autoimmunity. In this issue of theJCI, Pala and colleagues reveal that individuals with...