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https://www.readbyqxmd.com/read/28533268/the-transcription-factor-gli3-promotes-b-cell-development-in-fetal-liver-through-repression-of-shh
#1
Anisha Solanki, Ching-In Lau, José Ignacio Saldaña, Susan Ross, Tessa Crompton
Before birth, B cells develop in the fetal liver (FL). In this study, we show that Gli3 activity in the FL stroma is required for B cell development. In the Gli3-deficient FL, B cell development was reduced at multiple stages, whereas the Sonic hedgehog (Hh [Shh])-deficient FL showed increased B cell development, and Gli3 functioned to repress Shh transcription. Use of a transgenic Hh-reporter mouse showed that Shh signals directly to developing B cells and that Hh pathway activation was increased in developing B cells from Gli3-deficient FLs...
May 22, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28502113/epigenetic-modifications-of-the-vh-region-after-djh-recombination-in-pro-b-cells
#2
Yanying Dong, Caijun Wu, Xiaohui Zhao, Ping Zhang, Hua Zhang, Mingzhe Zheng, Shichang Li, Junna Jiao, Xiaozhuo Yu, Zhuangwei Lv, Yanhong Ji
The variable region of murine immunoglobulin heavy chain (Igh) is assembled by sequential DH -JH and VH -DJH recombination. The accessibility of Igh locus determines the order of rearrangement. Because of the large number of VH genes and lack of a suitable model, the epigenetic modifications of VH genes after DJH recombination have not previously been characterized. Here, we employed two v-Abl pro-B cell lines, in which Igh locus is in germline and DJH -recombined configuration, respectively. The DJH junction displays the characteristics of recombination center, such as high levels of activation-associated histone modifications and recombination activating gene protein (RAG) binding in DJH -rearranged pro-B cells, which extend the recombination center model proposed for germline Igh locus...
May 14, 2017: Immunology
https://www.readbyqxmd.com/read/28487787/ebv-negative-monomorphic-b-cell-posttransplant-lymphoproliferative-disorder-with-marked-morphologic-pleomorphism-and-pathogenic-mutations-in-asxl1-bcor-cdkn2a-nf1-and-tp53
#3
Agata M Bogusz
Posttransplant lymphoproliferative disorders (PTLDs) are a diverse group of lymphoid or plasmacytic proliferations frequently driven by Epstein-Barr virus (EBV). EBV-negative PTLDs appear to represent a distinct entity. This report describes an unusual case of a 33-year-old woman that developed a monomorphic EBV-negative PTLD consistent with diffuse large B-cell lymphoma (DLBCL) 13 years after heart-lung transplant. Histological examination revealed marked pleomorphism of the malignant cells including nodular areas reminiscent of classical Hodgkin lymphoma (cHL) with abundant large, bizarre Hodgkin-like cells...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28484276/tumor-protein-53-mutations-are-enriched-in-diffuse-large-b-cell-lymphoma-with-irregular-cd19-marker-expression
#4
Marina Kazantseva, Noelyn A Hung, Sunali Mehta, Imogen Roth, Ramona Eiholzer, Alison M Rich, Benedict Seo, Margaret A Baird, Antony W Braithwaite, Tania L Slatter
Accumulating evidence suggests tumor protein 53 (p53) promotes correct cellular differentiation. Thus, mutant TP53 may be more frequent in tumors with irregular differentiation. This study investigated whether TP53 mutations were more frequent in diffuse large B cell lymphoma (DLBCL) that lacked the B cell lineage marker CD19. Sixteen CD19 negative and 78 CD19 positive DLBCL were sequenced for TP53 mutations. Twenty nine tumors had TP53 mutations and were associated with poorer survival. Mutant TP53 was more frequent in CD19 negative lymphomas (81% versus 21%, p < 0...
May 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28476192/gene-expression-profiling-of-acute-lymphoblastic-leukemia-in-children-with%C3%A2-very-early-relapse
#5
Juan Carlos Núñez-Enríquez, Diego Alberto Bárcenas-López, Alfredo Hidalgo-Miranda, Elva Jiménez-Hernández, Vilma Carolina Bekker-Méndez, Janet Flores-Lujano, Karina Anastacia Solis-Labastida, Gabriela Bibiana Martínez-Morales, Fausto Sánchez-Muñoz, Laura Eugenia Espinoza-Hernández, Martha Margarita Velázquez-Aviña, Laura Elizabeth Merino-Pasaye, Alejandra Jimena García Velázquez, María Luisa Pérez-Saldívar, Raúl Mojica-Espinoza, Julián Ramírez-Bello, Silvia Jiménez-Morales, Juan Manuel Mejía-Aranguré
BACKGROUND AND AIMS: Acute lymphoblastic leukemia (ALL) is the most common childhood cancer worldwide. Mexican patients have high mortality rates, low frequency of good prognosis biomarkers (i.e., ETV6-RUNX1) and a high proportion is classified at the time of diagnosis with a high risk to relapse according to clinical features. In addition, very early relapses are more frequently observed than in other populations. The aim of the study was to identify new potential biomarkers associated with very early relapse in Mexican ALL children through transcriptome analysis...
November 2016: Archives of Medical Research
https://www.readbyqxmd.com/read/28463873/transcriptional-circuits-in-b-cell-transformation
#6
Yeguang Hu, Toshimi Yoshida, Katia Georgopoulos
PURPOSE OF REVIEW: Loss of IKAROS in committed B cell precursors causes a block in differentiation while at the same time augments aberrant cellular properties, such as bone marrow stromal adhesion, self-renewal and resistance to glucocorticoid-mediated cell death. B cell acute lymphoblastic leukaemias originating from these early stages of B cell differentiation and associated with IKAROS mutations share a high-risk cellular phenotype suggesting that deregulation of IKAROS-based mechanisms cause a highly malignant disease process...
April 29, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28446319/-progress-on-treatment-of-splenic-marginal-zone-lymphoma-review
#7
Ling Wu, Chen Tian, Yi-Zhuo Zhang
Splenic marginal zone lymphoma (SMZL) is an uncommon indolent B-cell non-Hodgkin's lymphoma. The clinical features of SMZL are splenomegaly, lymphocytosis and cytopenia in peripheral blood. Immunopheno-typically, the neoplastic cells are typically positive for CD45, CD20, CD79a, PAX5, IgD, BCL-2. The previous common used treatment is splenectomy and chemotherapy. Nowadays, rituximab-based chemotherapy regimens has improved the curative effect dramaticlly. IFN-α with or without ribavirin can be used to treat the patients with HCV infection...
April 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28420429/deptor-maintains-plasma-cell-differentiation-and-favorably-affects-prognosis-in-multiple-myeloma
#8
Dalia Quwaider, Luis A Corchete, Irena Misiewicz-Krzeminska, María E Sarasquete, José J Pérez, Patryk Krzeminski, Noemí Puig, María Victoria Mateos, Ramón García-Sanz, Ana B Herrero, Norma C Gutiérrez
BACKGROUND: The B cell maturation process involves multiple steps, which are controlled by relevant pathways and transcription factors. The understanding of the final stages of plasma cell (PC) differentiation could provide new insights for therapeutic strategies in multiple myeloma (MM). Here, we explore the role of DEPTOR, an mTOR inhibitor, in the terminal differentiation of myeloma cells, and its potential impact on patient survival. METHODS: The expression level of DEPTOR in MM cell lines and B cell populations was measured by real-time RT-PCR, and/or Western blot analysis...
April 18, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28418282/immunohistochemical-detection-of-hairy-cell-leukemia-in-paraffin-sections-the-role-of-pax5-and-cd103-double-staining-to-improve-specificity-and-sensitivity
#9
Igor B Rozenvald, Maurice D Richardson, Lenessa Brock, Russell L Maiese
CONTEXT: - In hematopathology practice, abnormal expression of CD103 on B cells is detected by flow cytometry in hairy cell leukemia (HCL) and, in combination with other phenotypic and morphologic findings, provides diagnostic specificity and sensitivity. Immunostaining on paraffin sections makes it possible to perform immunophenotyping in situ. However, normal bone marrow contains CD103-positive cells, which are not B cells, making it difficult to be certain about low-level involvement by HCL...
April 18, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28401483/molecular-profiling-of-gene-copy-number-abnormalities-in-key-regulatory-genes-in-high-risk-b-lineage-acute-lymphoblastic-leukemia-frequency-and-their-association-with-clinicopathological-findings-in-indian-patients
#10
Prerana Bhandari, Firoz Ahmad, Bibhu Ranjan Das
Genes related to key cellular pathways are frequently altered in B cell ALL and are associated with poor survival especially in high-risk (HR) subgroups. We examined gene copy number abnormalities (CNA) in 101 Indian HR B cell ALL patients and their correlation with clinicopathological features by multiplex ligation-dependent probe amplification. Overall, CNA were detected in 59 (59%) cases, with 26, 10 and 23% of cases harboring 1, 2 or +3 CNA. CNA were more prevalent in BCR-ABL1 (60%), pediatric (64%) and high WCC (WBC count) (63%) patients...
May 2017: Medical Oncology
https://www.readbyqxmd.com/read/28390747/fcrl4-b-cells-in-salivary-glands-of-primary-sj%C3%A3-gren-s-syndrome-patients
#11
Erlin A Haacke, Hendrika Bootsma, Fred K L Spijkervet, Annie Visser, Arjan Vissink, Philip M Kluin, Frans G M Kroese
Fc receptor-like protein 4 (FcRL4) is normally expressed on a small subset of mucosa-associated B-cells, as well as on mucosa-associated lymphoid tissue (MALT) lymphoma B-cells. Primary Sjögren's syndrome (pSS) patients have an increased risk of developing MALT lymphomas, preferentially in the parotid glands. For this reason we studied here by immunohistochemistry and mRNA analysis whether FcRL4 expressing B-cells are present in salivary gland tissue (labial and parotid) of pSS patients (n = 54) and non-pSS sicca patients (n = 16) and whether parotid gland MALT lymphomas in pSS patients (n = 49) also express this receptor...
April 5, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28382853/primary-cerebellar-lymphoma-with-hodgkin-lymphoma-like-morphology-in-a-cat
#12
Yuka Yoshino, James K Chambers, Taichi Nakamori, Yuko Goto-Koshino, Kazuo Nishigaki, Hajime Tsujimoto, Naoaki Matsuki, Hiroyuki Nakayama, Kazuyuki Uchida
A 4-y-old cat exhibited neurologic signs such as wobbling, right head tilt, and intention tremor, and MRI revealed a mass in the cerebellum. The cat died 5 mo after initial presentation, and no neoplastic lesions, other than the cerebellar mass, were observed at autopsy. Histologically, large atypical cells resembling Hodgkin cells, with single large inclusion-like nucleoli, and those resembling Reed-Sternberg cells, with symmetrically arranged nuclei, had infiltrated the left side of the cerebellum and were admixed with small lymphocytes...
April 1, 2017: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/28371317/molecular-characterization-of-acute-lymphoblastic-leukemia-with-high-crlf2-gene-expression-in-childhood
#13
Juliane Schmäh, Birthe Fedders, Renate Panzer-Grümayer, Susanna Fischer, Martin Zimmermann, Elif Dagdan, Susanne Bens, Denis Schewe, Anja Moericke, Julia Alten, Kirsten Bleckmann, Reiner Siebert, Martin Schrappe, Martin Stanulla, Gunnar Cario
BACKGROUND: A high-level expression of the CRLF2 gene is frequent in precursor B-cell acute lymphoblastic leukemia (pB-ALL) and can be caused by different genetic aberrations. The presence of the most frequent alteration, the P2RY8/CRLF2 fusion, was shown to be associated with a high relapse incidence in children treated according to ALL-Berlin-Frankfurt-Münster (BFM) protocols, which is poorly understood. Moreover, the frequency of other alterations has not been systematically analyzed yet...
April 1, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28369050/antagonism-of-b-cell-enhancer-networks-by-stat5-drives-leukemia-and-poor-patient-survival
#14
Casey D S Katerndahl, Lynn M Heltemes-Harris, Mark J L Willette, Christine M Henzler, Seth Frietze, Rendong Yang, Hilde Schjerven, Kevin A T Silverstein, Laura B Ramsey, Gregory Hubbard, Andrew D Wells, Roland P Kuiper, Blanca Scheijen, Frank N van Leeuwen, Markus Müschen, Steven M Kornblau, Michael A Farrar
The transcription factor STAT5 has a critical role in B cell acute lymphoblastic leukemia (B-ALL). How STAT5 mediates this effect is unclear. Here we found that activation of STAT5 worked together with defects in signaling components of the precursor to the B cell antigen receptor (pre-BCR), including defects in BLNK, BTK, PKCβ, NF-κB1 and IKAROS, to initiate B-ALL. STAT5 antagonized the transcription factors NF-κB and IKAROS by opposing regulation of shared target genes. Super-enhancers showed enrichment for STAT5 binding and were associated with an opposing network of transcription factors, including PAX5, EBF1, PU...
April 3, 2017: Nature Immunology
https://www.readbyqxmd.com/read/28346666/disseminated-peripheral-neuroblastoma-in-a-rhodesian-ridgeback-dog
#15
R W Cook, L A Abraham, C I McCowan
CASE REPORT: A 4-year-old neutered male Rhodesian Ridgeback dog with right-sided Horner's syndrome, bilateral laryngeal paralysis, neck pain and bilateral hindlimb ataxia was euthanased following deterioration of its neurological status. Necropsy examination revealed an off-white retropharyngeal neoplastic mass (100 × 30 × 30 mm) attached to the base of the skull on the right side and macroscopic nodular metastases in the spleen and three vertebral bodies (C6, C7 and T6), including a nodule attached to the dura at C7...
April 2017: Australian Veterinary Journal
https://www.readbyqxmd.com/read/28316978/identification-of-significant-pathways-induced-by-pax5-haploinsufficiency-based-on-protein-protein-interaction-networks-and-cluster-analysis-in-raji-cell-line
#16
Jia Gu, TongJuan Li, Lei Zhao, Xue Liang, Xing Fu, Jue Wang, Zhen Shang, Wei Huang, Jianfeng Zhou
PAX5 encodes a transcription factor essential for B-cell differentiation, and PAX5 haploinsufficiency is involved in tumorigenesis. There were few studies on how PAX5 haploinsufficiency regulated genes expression to promote tumorigenesis. In this study, we constructed the cell model of PAX5 haploinsufficiency using gene editing technology in Raji cells, detected differentially expressed genes in PAX5 haploinsufficiency Raji cells, and used protein-protein interaction networks and cluster analysis to comprehensively investigate the cellular pathways involved in PAX5 haploinsufficiency...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28302063/epigenetic-and-genetic-alterations-and-their-influence-on-gene-regulation-in-chronic-lymphocytic-leukemia
#17
Di Huang, Ivan Ovcharenko
BACKGROUND: To understand the changes of gene regulation in carcinogenesis, we explored signals of DNA methylation - a stable epigenetic mark of gene regulatory elements - and designed a computational model to profile loss and gain of regulatory elements (REs) during carcinogenesis. We also utilized sequencing data to analyze the allele frequency of single nucleotide polymorphisms (SNPs) and detected the cancer-associated SNPs, i.e., the SNPs displaying the significant allele frequency difference between cancer and normal samples...
March 16, 2017: BMC Genomics
https://www.readbyqxmd.com/read/28297581/systemic-delivery-of-bone-marrow-mesenchymal-stem-cells-for-in-situ-intervertebral-disc-regeneration
#18
Carla Cunha, Catarina R Almeida, Maria Inês Almeida, Andreia M Silva, Maria Molinos, Sofia Lamas, Catarina L Pereira, Graciosa Q Teixeira, António T Monteiro, Susana G Santos, Raquel M Gonçalves, Mário A Barbosa
Cell therapies for intervertebral disc (IVD) regeneration presently rely on transplantation of IVD cells or stem cells directly to the lesion site. Still, the harsh IVD environment, with low irrigation and high mechanical stress, challenges cell administration and survival. In this study, we addressed systemic transplantation of allogeneic bone marrow mesenchymal stem cells (MSCs) intravenously into a rat IVD lesion model, exploring tissue regeneration via cell signaling to the lesion site. MSC transplantation was performed 24 hours after injury, in parallel with dermal fibroblasts as a control; 2 weeks after transplantation, animals were killed...
March 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28221211/a-rare-case-of-ck20-ck7-double-negative-merkel-cell-carcinoma
#19
Mary D Le, Luke H OʼSteen, David S Cassarino
The authors present a case of Merkel cell carcinoma (MCC) with unique immunohistochemical staining characteristics. A 57-year-old woman presented with a firm 0.3 cm tan papule on her left nasal-labial fold that was reportedly increasing in size and bleeding. She had a history of multiple head and neck actinic keratoses, papillary thyroid carcinoma, and a family history of an uncle with melanoma. The clinical differential diagnosis was "non-melanoma skin cancer." Histological examination showed a markedly atypical-appearing basaloid neoplasm, present mostly in the dermis, with focal pagetoid spread into the epidermis...
March 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28219927/molecular-role-of-the-pax5-etv6-oncoprotein-in-promoting-b-cell-acute-lymphoblastic-leukemia
#20
Leonie Smeenk, Maria Fischer, Sabine Jurado, Markus Jaritz, Anna Azaryan, Barbara Werner, Mareike Roth, Johannes Zuber, Martin Stanulla, Monique L den Boer, Charles G Mullighan, Sabine Strehl, Meinrad Busslinger
PAX5 is a tumor suppressor in B-ALL, while the role of PAX5 fusion proteins in B-ALL development is largely unknown. Here, we studied the function of PAX5-ETV6 and PAX5-FOXP1 in mice expressing these proteins from the Pax5 locus. Both proteins arrested B-lymphopoiesis at the pro-B to pre-B-cell transition and, contrary to their proposed dominant-negative role, did not interfere with the expression of most regulated Pax5 target genes. Pax5-Etv6, but not Pax5-Foxp1, cooperated with loss of the Cdkna2a/b tumor suppressors in promoting B-ALL development...
March 15, 2017: EMBO Journal
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