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https://www.readbyqxmd.com/read/28337270/n-wasp-promotes-invasion-and-migration-of-cervical-cancer-cells-through-regulating-p38-mapks-signaling-pathway
#1
Jinxuan Hou, Hui Yang, Xin Huang, Xiaohua Leng, Fuxiang Zhou, Conghua Xie, Yunfeng Zhou, Yu Xu
Neural Wiskott-Aldrich syndrome protein (N-WASP) is an important member of the WASP family involved in the actin cytoskeleton reorganization. Recent evidence suggests that N-WASP may play important roles in tumor progression and metastasis. However, the contribution of N-WASP to cervical cancer is still unknown. The present study focused on elucidating the role of N-WASP in the malignant behavior of cervical cancer cells. We found that N-WASP overexpressed in cervical cancer tissues compared with paired paracancerous tissues and normal tissues, and similar results were observed in several cervical cancer cell lines...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28333714/novel-regulators-of-plasma-lipid-levels
#2
Natalia Loaiza, Federico Oldoni, Jan A Kuivenhoven
PURPOSE OF REVIEW: To highlight very recent studies identifying novel regulatory molecules and mechanisms in plasma lipid metabolism. RECENT FINDINGS: Two novel regulatory mechanisms of LDL receptor (LDLR) intracellular trafficking have been described. The "COMMD/CCDC22/CCDC93" and "Wiskott-Aldrich syndrome protein and SCAR homologue" complexes were found to be involved in LDLR endosomal sorting and recycling, whereas the GRP94 was shown to protect LDLR from early degradation within the hepatocyte secretory pathway...
March 22, 2017: Current Opinion in Lipidology
https://www.readbyqxmd.com/read/28332566/a-conformational-change-within-the-wave2-complex-regulates-its-degradation-following-cellular-activation
#3
Noah Joseph, Guy Biber, Sophia Fried, Barak Reicher, Omer Levy, Batel Sabag, Elad Noy, Mira Barda-Saad
WASp family Verprolin-homologous protein-2 (WAVE2), a member of the Wiskott-Aldrich syndrome protein (WASp) family of actin nucleation promoting factors, is a central regulator of actin cytoskeleton polymerization and dynamics. Multiple signaling pathways operate via WAVE2 to promote the actin-nucleating activity of the actin-related protein 2/3 (Arp2/3) complex. WAVE2 exists as a part of a pentameric protein complex known as the WAVE regulatory complex (WRC), which is unstable in the absence of its individual proteins...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28296961/correction-gaussian-binary-restricted-boltzmann-machines-for-modeling-natural-image-statistics
#4
Jan Melchior, Nan Wang, Laurenz Wiskott
[This corrects the article DOI: 10.1371/journal.pone.0171015.].
2017: PloS One
https://www.readbyqxmd.com/read/28287640/successful-hla-haploidentical-hsct-with-post-transplant-cyclophosphamide-in-wiskott-aldrich-syndrome
#5
P Kreetapirom, S Hongeng, W Manuyakorn, U Anurathapan, S Pakakasama, N Sirachainan, B S Andersson
No abstract text is available yet for this article.
March 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28270364/new-frontiers-in-the-therapy-of-primary-immunodeficiency-from-gene-addition-to-gene-editing
#6
REVIEW
Donald B Kohn, Caroline Y Kuo
The most severe primary immune deficiency diseases (PIDs) have been successfully treated with allogeneic hematopoietic stem cell transplantation for more than 4 decades. However, such transplantations have the best outcomes when there is a well-matched donor available because immune complications, such as graft-versus-host disease, are greater without a matched sibling donor. Gene therapy has been developed as a method to perform autologous transplantations of a patient's own stem cells that are genetically corrected...
March 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28261277/gene-therapy-for-primary-immune-deficiencies-a-canadian-perspective
#7
REVIEW
Xiaobai Xu, Chetankumar S Tailor, Eyal Grunebaum
The use of gene therapy (GT) for the treatment of primary immune deficiencies (PID) including severe combined immune deficiency (SCID) has progressed significantly in the recent years. In particular, long-term studies have shown that adenosine deaminase (ADA) gene delivery into ADA-deficient hematopoietic stem cells that are then transplanted into the patients corrects the abnormal function of the ADA enzyme, which leads to immune reconstitution. In contrast, the outcome was disappointing for patients with X-linked SCID, Wiskott-Aldrich syndrome and chronic granulomatous disease who received GT followed by autologous gene corrected transplantations, as many developed hematological malignancies...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28255019/a-risk-factor-analysis-of-outcomes-after-unrelated-cord-blood-transplantation-for-children-with-wiskott-aldrich-syndrome
#8
Zhanna Shekhovtsova, Carmem Bonfim, Annalisa Ruggeri, Samantha Nichele, Kristin Page, Amal AlSeraihy, Francisco Barriga, José Sánchez de Toledo Codina, Paul Veys, Jaap Jan Boelens, Karin Mellgren, Henrique Bittencourt, Tracey O' Brien, Peter J Shaw, Alicja Chybicka, Fernanda Volt, Federica Giannotti, Eliane Gluckman, Joanne Kurtzberg, Andrew R Gennery, Vanderson Rocha
Wiskott-Aldrich syndrome is a severe X-linked recessive immune deficiency disorder. A scoring system of Wiskott-Aldrich syndrome severity (0.5-5) distinguishes 2 phenotypes: X-linked thrombocytopenia and classic Wiskott-Aldrich syndrome. Hematopoietic cell transplantation is curative for Wiskott-Aldrich syndrome, however the use of unrelated umbilical cord blood transplantation has seldom been described. We analyzed umbilical cord blood transplantation outcomes for 90 patients. Median age at umbilical cord blood transplantation was 1...
March 2, 2017: Haematologica
https://www.readbyqxmd.com/read/28244429/tin-x-o-y-coatings-facilitate-the-initial-adhesion-of-osteoblasts-to-create-a-suitable-environment-for-their-proliferation-and-the-recruitment-of-endothelial-cells
#9
M Moussa, O Banakh, B Wehrle-Haller, P Fontana, S Scherrer, M Cattani, A Wiskott, S Durual
Titanium-nitride-oxide coatings (TiN x O y ) improve osseointegration of endosseous implants. The exact mechanisms by which these effects are mediated are poorly understood except for an increase of osteoblast proliferation while a high degree of differentiation is maintained. One hypothesis holds that TiN x O y facilitates the initial spreading and adhesion of the osteoblasts. The aim of this work was to investigate the molecular mechanisms of osteoblast adhesion on TiN x O y as compared to microrough titanium SLA...
February 28, 2017: Biomedical Materials
https://www.readbyqxmd.com/read/28212567/ribosomal-dna-status-inferred-from-dna-cloud-assays-and-mass-spectrometry-identification-of-agarose-squeezed-proteins-interacting-with-chromatin-aspic-ms
#10
Kamil Krol, Justyna Jendrysek, Janusz Debski, Marek Skoneczny, Anna Kurlandzka, Joanna Kaminska, Michal Dadlez, Adrianna Skoneczna
Ribosomal RNA-encoding genes (rDNA) are the most abundant genes in eukaryotic genomes. To meet the high demand for rRNA, rDNA genes are present in multiple tandem repeats clustered on a single or several chromosomes and are vastly transcribed. To facilitate intensive transcription and prevent rDNA destabilization, the rDNA-encoding portion of the chromosome is confined in the nucleolus. However, the rDNA region is susceptible to recombination and DNA damage, accumulating mutations, rearrangements and atypical DNA structures...
February 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28203325/inherited-thrombocytopenia-with-a-different-type-of-gene-mutation-a-brief-literature-review-and-two-case-studies
#11
Mohammad Taghi Arzanian
Hereditary thrombocytopenias are rare bleeding disorders, which cause a deficiency of platelets in early infancy. This group of disorders is sometimes associated with abnormal phenotypes, like absence of radius. Diagnosis of this type of thrombocytopenia is usually difficult; other causes of thrombocytopenia, such as immune disorders and infections, must be ruled out. The symptoms of hereditary thrombocytopenia also vary from seldom and mild to severe bleeding and occasionally may first occur in late childhood...
October 2016: Iranian Journal of Pediatrics
https://www.readbyqxmd.com/read/28202128/-wiskott-aldrich-syndrome-with-special-phenotypes-report-of-3-cases
#12
Jian-Xin He, Guan-Rong Chen, Bao-Ping Xu, Xiu-Yun Liu, Jin-Gang Gui, Kun-Ling Shen, Zai-Fang Jiang, Yu-Lung Lau
No abstract text is available yet for this article.
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28183734/skap2-is-required-for-%C3%AE-2-integrin-mediated-neutrophil-recruitment-and-functions
#13
Mark Boras, Stephanie Volmering, Arne Bokemeyer, Jan Rossaint, Helena Block, Bernadette Bardel, Veerle Van Marck, Barbara Heitplatz, Stefanie Kliche, Annegret Reinhold, Clifford Lowell, Alexander Zarbock
Integrin activation is required for neutrophil functions. Impaired integrin activation on neutrophils is the hallmark of leukocyte adhesion deficiency (LAD) syndrome in humans, characterized by impaired leukocyte recruitment and recurrent infections. The Src kinase-associated phosphoprotein 2 (Skap2) is involved in integrin functions in different leukocyte subtypes. However, the role of Skap2 in β2 integrin activation and neutrophil recruitment is unknown. In this study, we demonstrate the crucial role of Skap2 in regulating actin polymerization and binding of talin-1 and kindlin-3 to the β2 integrin cytoplasmic domain, thereby being indispensable for β2 integrin activation and neutrophil recruitment...
March 6, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28152552/gaussian-binary-restricted-boltzmann-machines-for-modeling-natural-image-statistics
#14
Jan Melchior, Nan Wang, Laurenz Wiskott
We present a theoretical analysis of Gaussian-binary restricted Boltzmann machines (GRBMs) from the perspective of density models. The key aspect of this analysis is to show that GRBMs can be formulated as a constrained mixture of Gaussians, which gives a much better insight into the model's capabilities and limitations. We further show that GRBMs are capable of learning meaningful features without using a regularization term and that the results are comparable to those of independent component analysis. This is illustrated for both a two-dimensional blind source separation task and for modeling natural image patches...
2017: PloS One
https://www.readbyqxmd.com/read/28129868/50-years-ago-in-the-journal-of-pediatrics-wiskott-aldrich-syndrome-clinical-immunologic-and-pathologic-observations
#15
Emily B Heikamp
No abstract text is available yet for this article.
February 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28129089/activation-of-compensatory-pathways-via-rac2-in-the-absence-of-the-cdc42-effector-wiskott-aldrich-syndrome-protein-in-dendritic-cells
#16
Marisa A P Baptista, Lisa S Westerberg
There is extensive crosstalk between different Rho GTPases, including Cdc42, Rac1, and Rac2, and they can activate or inhibit the activity of each other. Dendritic cells express both Rac1 and Rac2. Due to posttranslational modification of lipid anchors, Rac1 localizes mainly to the plasma membrane whereas Rac2 localizes to the phagosomal membrane where it assembles the NADPH complex. Our recent study of primary immunodeficiency disease caused by mutations in the Cdc42 effector Wiskott-Aldrich syndrome protein (WASp) has shed light on the compensatory mechanisms between Rho GTPases and their effector proteins...
January 27, 2017: Small GTPases
https://www.readbyqxmd.com/read/28115704/wave1-in-neurons-expressing-the-d1-dopamine-receptor-regulates-cellular-and-behavioral-actions-of-cocaine
#17
Ilaria Ceglia, Ko-Woon Lee, Michael E Cahill, Steven M Graves, David Dietz, Dalton J Surmeier, Eric J Nestler, Angus C Nairn, Paul Greengard, Yong Kim
Wiskott-Aldrich syndrome protein (WASP) family verprolin homologous protein 1 (WAVE1) regulates actin-related protein 2/3 (Arp2/3) complex-mediated actin polymerization. Our previous studies have found WAVE1 to be inhibited by Cdk5-mediated phosphorylation in brain and to play a role in the regulation of dendritic spine morphology. Here we report that mice in which WAVE1 was knocked out (KO) in neurons expressing the D1 dopamine receptor (D1-KO), but not mice where WAVE1 was knocked out in neurons expressing the D2 dopamine receptor (D2-KO), exhibited a significant decrease in place preference associated with cocaine...
February 7, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28106075/phosphatidylinositol-3-4-bisphosphate-regulates-neurite-initiation-and-dendrite-morphogenesis-via-actin-aggregation
#18
Shu-Xin Zhang, Li-Hui Duan, Shun-Ji He, Gui-Feng Zhuang, Xiang Yu
Neurite initiation is critical for neuronal morphogenesis and early neural circuit development. Recent studies showed that local actin aggregation underneath the cell membrane determined the site of neurite initiation. An immediately arising question is what signaling mechanism initiated actin aggregation. Here we demonstrate that local clustering of phosphatidylinositol 3,4-bisphosphate (PI(3,4)P2), a phospholipid with relatively few known signaling functions, is necessary and sufficient for aggregating actin and promoting neuritogenesis...
February 2017: Cell Research
https://www.readbyqxmd.com/read/28099198/osteomyelitis-of-the-navicular-bone-a-case-report
#19
Nobuyuki Kumahashi, Suguru Kuwata, Shinji Imade, Yuji Uchio
A 16-year-old boy developed left foot pain of unknown cause that was unresponsive to conservative treatment, associated with fever and difficulty walking. He was admitted to our hospital with osteomyelitis of the accessory and body of the navicular bone. Surgery could not be performed because the patient had been diagnosed with Wiskott-Aldrich syndrome. After antibiotic therapy, laboratory abnormalities and pain had resolved. One year after treatment, the patient had returned to his original level of sports activity...
January 17, 2017: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/28089394/grinding-damage-assessment-for-cad-cam-restorative-materials
#20
Philippe Curran, Maria Cattani-Lorente, H W Anselm Wiskott, Stéphane Durual, Susanne S Scherrer
OBJECTIVES: To assess surface/subsurface damage after grinding with diamond discs on five CAD-CAM restorative materials and to estimate potential losses in strength based on crack size measurements of the generated damage. METHODS: The materials tested were: Lithium disilicate (LIT) glass-ceramic (e.max CAD), leucite glass-ceramic (LEU) (Empress CAD), feldspar ceramic (VM2) (Vita Mark II), feldspar ceramic-resin infiltrated (EN) (Enamic) and a composite reinforced with nano ceramics (LU) (Lava Ultimate)...
January 12, 2017: Dental Materials: Official Publication of the Academy of Dental Materials
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