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https://www.readbyqxmd.com/read/29773598/concomitant-wt1-mutations-predict-poor-prognosis-in-acute-myeloid-leukemia-patients-with-double-mutant-cebpa
#1
Feng-Ming Tien, Hsin-An Hou, Jih-Luh Tang, Yuan-Yeh Kuo, Chien-Yuan Chen, Cheng-Hong Tsai, Ming Yao, Chien-Ting Lin, Chi-Cheng Li, Shang-Yi Huang, Bor-Sheng Ko, Szu-Chun Hsu, Shang-Ju Wu, Jia-Hau Liu, Sheng-Chieh Chou, Woei Tsay, Mei-Hsuan Tseng, Ming-Chih Liu, Chia-Wen Liu, Liang-In Lin, Wen-Chien Chou, Hwei-Fang Tien
No abstract text is available yet for this article.
May 17, 2018: Haematologica
https://www.readbyqxmd.com/read/29766535/deceptively-bland-cutaneous-angiosarcoma-on-the-nose-mimicking-hemangioma-a-clinicopathologic-and-immunohistochemical-analysis
#2
Christina Mitteldorf, Mar Llamas-Velasco, Hans-Joachim Schulze, Kai-Martin Thoms, Thomas Mentzel, Michael Tronnier, Heinz Kutzner
BACKGROUND: We investigated two cases of deceptively bland cutaneous angiosarcoma, which showed a uniform clinical presentation with a rapidly growing tumor on the nose. It remains unclear whether this was a primary cutaneous manifestation or a metastasis. Both tumors initially presented a high histologic overlap with a benign vascular tumor. The diagnosis was primarily based on the rapidly progressing clinical course and on the results of the staging procedures. METHODS: Immunohistochemical stains were performed for cytokeratin (AE1/AE3 and MNF116), CD31, ERG, CD34(HPCA1/my10), D2-40/podoplanin, LYVE-1, Ki67, PHH3, αSMA(1A4), MYC, FOS-B, CAMTA-1, TFE-3, WT1, nestin, VEGFR-2(KDR), VEGFR-3(FLT4), HHV8...
May 15, 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29764980/interplay-of-cell-cell-contacts-and-rhoa-mrtf-a-signaling-regulates-cardiomyocyte-identity
#3
Tatjana Dorn, Jessica Kornherr, Elvira I Parrotta, Dorota Zawada, Harold Ayetey, Gianluca Santamaria, Laura Iop, Elisa Mastantuono, Daniel Sinnecker, Alexander Goedel, Ralf J Dirschinger, Ilaria My, Svenja Laue, Tarik Bozoglu, Christian Baarlink, Tilman Ziegler, Elisabeth Graf, Rabea Hinkel, Giovanni Cuda, Stefan Kääb, Andrew A Grace, Robert Grosse, Christian Kupatt, Thomas Meitinger, Austin G Smith, Karl-Ludwig Laugwitz, Alessandra Moretti
Cell-cell and cell-matrix interactions guide organ development and homeostasis by controlling lineage specification and maintenance, but the underlying molecular principles are largely unknown. Here, we show that in human developing cardiomyocytes cell-cell contacts at the intercalated disk connect to remodeling of the actin cytoskeleton by regulating the RhoA-ROCK signaling to maintain an active MRTF/SRF transcriptional program essential for cardiomyocyte identity. Genetic perturbation of this mechanosensory pathway activates an ectopic fat gene program during cardiomyocyte differentiation, which ultimately primes the cells to switch to the brown/beige adipocyte lineage in response to adipogenesis-inducing signals...
May 15, 2018: EMBO Journal
https://www.readbyqxmd.com/read/29757709/how-to-assess-the-best-immunohistochemical-panel-in-the-diagnosis-of-malignant-pleural-mesothelioma-in-a-pathology-lab
#4
Mona Mlika, Oumeima Lamzibri, Saoussen Bacha, Soumeya Laabidi, Chokri Haddouchi, Faouzi El Mezni
BACKGROUND: malignant pleural mesothelioma (MPM) is a rare tumor with a challenging diagnosis. Even if, clinical data are mandatory to suspect the diagnosis, the positive diagnosis is based on microscopic features. Morphologic features are still the port of call of the diagnosis but their non specific character and the multiplicity of differential diagnoses made the immunohistochemical markers mandatory for the diagnosis. Many antibodies with a positive diagnostic value including claretinin, mesothelin, WT1 and antibodies with a negative diagnostic value including TTF1, EMA, CD15 are recommended by the scientific societies...
May 14, 2018: Journal of Immunoassay & Immunochemistry
https://www.readbyqxmd.com/read/29753838/wilms-tumor-1-wt1-expression-using-a-standardized-european-leukemia-net-certified-assay-compared-to-other-methods-for-detection-of-minimal-residual-disease-in-mds-and-aml-patients-after-allogeneic-blood-stem-cell-transplantation
#5
Christina Rautenberg, Sabrina Pechtel, Barbara Hildebrandt, Beate Betz, Ariane Dienst, Kathrin Nachtkamp, Mustafa Kondakci, Stefanie Geyh, Dagmar Wieczorek, Rainer Haas, Ulrich Germing, Guido Kobbe, Thomas Schroeder
Overexpressed Wilms' Tumor 1 (WT1) gene is informative in many patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS) and is measurable in peripheral blood (PB). Despite these advantages WT1 has not broadly been established as marker for minimal residual disease (MRD) monitoring after allogeneic transplantation (allo-HSCT) due to limited patient numbers, differing sample sources and non-standardized in-house methods. To estimate its value as MRD marker we serially quantified PB WT1 expression using a standardized European Leukemia Net-certified assay in 59 patients with AML and MDS after allo-HSCT...
May 10, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29748075/protective-effect-of-calcitriol-on-podocytes-in-spontaneously-hypertensive-rat
#6
Weiwei Shi, Ling Guo, Guangyi Liu, Tao Peng, Hongyu Li, Tingting Xie, Dengren Li, Junhui Zhen, Yan Wang, Huimin Yang, Xiangdong Yang
BACKGROUND: Hypertension is a major global public health issue. Uncontrolled hypertension leads to organ damage, especially renal damage. Calcitriol is used to treat osteoporosis, promote bone formation, and increase bone mass. Previous studies have demonstrated that 1,25(OH)2D3, in addition to its classic role, also has multiple immune regulation and renoprotective functions and inhibits the activity of the renin-angiotensin-aldosterone system (RASS). The aim of the current study was to investigate the renoprotective effects of calcitriol in a spontaneously hypertensive rat (SHR) model...
May 7, 2018: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/29743143/the-wilms-tumour-1-gene-as-a-factor-in-non-syndromic-hypospadias-evidence-and-controversy
#7
REVIEW
Gergely Buglyó, Dániel Beyer, Sándor Biró, Éva Oláh
Hypospadias is one of the most frequent congenital anomalies of the male external genitalia. Its pathogenesis is due to largely unknown or poorly understood genetic factors and is further complicated by environmental-intrauterine-risk factors. One of the genes currently in focus by molecular biologists and clinicians studying syndromic forms of hypospadias is the Wilms' tumour 1 (WT1) gene. There is controversy over whether WT1 defects are also responsible for isolated hypospadias. In this review, we briefly cover the role of WT1 as a transcription factor and discuss proposed pathogenic pathways leading to hypospadias, outlining possible directions for research...
May 7, 2018: Pathology
https://www.readbyqxmd.com/read/29739109/a-proposed-kinetic-model-for-the-diagnostic-and-prognostic-value-of-wt1-and-p53-in-acute-myeloid-leukemia
#8
Mohammad A Bani-Ahmad, Suleimman A Al-Sweedan, Mohammad A Al-Asseiri, Ahed J Alkhatib
BACKGROUND: Wilms tumor (WT1) and p53 proteins were identified in the pathogenesis of several malignancies, including hematological malignancies. As a result of their interaction and diverse context-specific functions, this study aimed to emphasize the diagnostic and prognostic impacts of WT1 and p53 expression in acute myeloid leukemia (AML). METHODS: Twelve bone marrow (BM) biopsies were obtained from AML patients who were diagnosed in accordance with the French-American-British diagnostic criteria...
March 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29738081/prame-peptide-specific-cd8-t-cells-represent-the-predominant-response-against-leukemia-associated-antigens-laas-in-healthy-individuals
#9
Sarah Matko, Julia Manderla, Maria Bonsack, Marc Schmitz, Martin Bornhauser, Torsten Tonn, Marcus Odendahl
Antigen-specific T cells isolated from healthy individuals (HIs) have shown great therapeutic potential upon adoptive transfer for the treatment of viremia in immunosuppressed patients. The lack of comprehensive data on the prevalence and characteristics of leukemia associated antigen (LAA)-specific T cells in HIs still limits such an approach for tumor therapy. Therefore, we have investigated T cell responses against prominent candidates comprising WT1, PRAME, Survivin, NY-ESO and p53 by screening PBMCs from HIs using intracellular IFN-γ staining following provocation with LAA peptide mixes...
May 8, 2018: European Journal of Immunology
https://www.readbyqxmd.com/read/29727824/chinese-and-europeans-with-acute-myeloid-leukemia-have-discordant-mutation-topographies
#10
Min Zhang, Jiawei Yin, Qinghua He, Fan Zhang, Hongyu Huang, Biao Wu, Xuedong Wang, Hong Liu, Hongchao Yin, Yan Zeng, Robert Peter Gale, Depei Wu, Bin Yin
Although the topography of mutations in persons of predominately European-descent with acute myeloid leukemia (AML) is well-described this is less so in Asians. We studied AML-related mutations in 289 consecutive Chinese (mostly Han) with newly-diagnosed de novo AML. Full-length coding sequence of NPM1 and CEBPA, IDH1 and IDH2 hotspot mutations and WT1 mutations in exons 7 and 9 were analyzed by PCR as were correlations with clinical and laboratory variables. CEBPA mutations were detected in 20% of subjects (95% confidence interval [CI] 15, 25%), NPM1 mutations in 20% (15, 25%), IDH1 mutations in 4% (1, 6%), IDH2 mutations in 11% (7, 15%) and WT1 mutations in 6% (3, 9%)...
April 22, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29708625/role-of-the-wilms-tumor-suppressor-gene-wt1-in-pancreatic-development
#11
Laura Ariza, Ana Cañete, Anabel Rojas, Ramón Muñoz-Chápuli, Rita Carmona
The Wilms tumor suppressor gene (Wt1) encodes a transcription factor involved in the development of a number of organs, but the role played by Wt1 in pancreatic development is unknown. The pancreas contains a population of pancreatic stellate cells (PSC) very important for pancreatic physiology. We described elsewhere that hepatic stellate cells originate from the WT1-expressing liver mesothelium. Thus, we checked if the origin of PSCs was similar. WT1 is expressed in the pancreatic mesothelium. Between E10...
April 30, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/29704660/genetic-and-epigenetic-control-of-adipose-development
#12
REVIEW
Olga Gulyaeva, Jon Dempersmier, Hei Sook Sul
White adipose tissue (WAT) is the primary energy storage organ and its excess contributes to obesity, while brown adipose tissue (BAT) and inducible thermogenic (beige/brite) adipocytes in WAT dissipate energy via Ucp1 to maintain body temperature. BAT and subcutaneous WAT develop perinatally while visceral WAT forms after birth from precursors expressing distinct markers, such as Myf5, Pref-1, Wt1, and Prx1, depending on the anatomical location. In addition to the embryonic adipose precursors, a pool of endothelial cells or mural cells expressing Pparγ, Pdgfrβ, Sma and Zfp423 may become adipocytes during WAT expansion in adults...
April 25, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29700887/cic-nutm1-fusion-a-case-which-expands-the-spectrum-of-nut-rearranged-epithelioid-malignancies
#13
Inga-Marie Schaefer, Paola Dal Cin, Christopher D M Fletcher, Glenn J Hanna, Christopher A French
NUT carcinoma (NC) shows very aggressive clinical behavior, occurs predominantly in the thorax and head and neck region of children and adults, and is defined by the presence of NUT (aka NUTM1) rearrangement, mostly BRD4-NUTM1 fusion resulting from t(15;19)(q13, p13.1). So-called "NUT variants" harbor alternate fusions between NUTM1 and BRD3, NSD3, ZNF532, or unknown partners. Rare cases of pediatric tumors with CIC-NUTM1 fusion were recently reported in somatic soft tissue, brain, and kidney. However, such cases have not been identified in adult patients and the presence of a fusion between CIC, characteristic of CIC-rearranged sarcoma, and NUTM1, a defining feature of NC - poses a diagnostic challenge...
April 26, 2018: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29695308/the-presence-of-human-mesenchymal-stem-cells-of-renal-origin-in-amniotic-fluid-increases-with-gestational-time
#14
Md Shaifur Rahman, Lucas-Sebastian Spitzhorn, Wasco Wruck, Carsten Hagenbeck, Percy Balan, Nina Graffmann, Martina Bohndorf, Audrey Ncube, Pascale V Guillot, Tanja Fehm, James Adjaye
BACKGROUND: Established therapies for managing kidney dysfunction such as kidney dialysis and transplantation are limited due to the shortage of compatible donated organs and high costs. Stem cell-based therapies are currently under investigation as an alternative treatment option. As amniotic fluid is composed of fetal urine harboring mesenchymal stem cells (AF-MSCs), we hypothesized that third-trimester amniotic fluid could be a novel source of renal progenitor and differentiated cells...
April 25, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29680194/the-challenge-of-evaluating-adnexal-masses-in-patients-with-breast-cancer
#15
REVIEW
Tomás Reinert, Angélica Nogueira-Rodrigues, Fabiola Procacci Kestelman, Patricia Ashton-Prolla, Márcia Silveira Graudenz, José Bines
This narrative literature review addresses the problem of an adnexal mass discovered during the course of breast cancer (BC) care, which may represent a benign condition, a metastatic process, or a primary ovarian cancer (OC), clinical scenarios associated with distinct physiopathology and prognosis. Furthermore, the coexistence of BC and OC in the same patient may be owing to a hereditary disorder, deserving specific management strategies and counseling. The initial detection and evaluation of an adnexal mass in a patient with BC requires a high index of suspicion, and the initial workup should include a thorough medical history and physical examination, measurement of tumor markers, complete blood count, and imaging tests...
March 12, 2018: Clinical Breast Cancer
https://www.readbyqxmd.com/read/29675416/free-floating-mesothelial-cells-in-pleural-fluid-after-lung-surgery
#16
Arne Kienzle, Andrew B Servais, Alexandra B Ysasi, Barry C Gibney, Cristian D Valenzuela, Willi L Wagner, Maximilian Ackermann, Steven J Mentzer
Objectives: The mesothelium, the surface layer of the heart, lung, bowel, liver, and tunica vaginalis, is a complex tissue implicated in organ-specific diseases and regenerative biology; however, the mechanism of mesothelial repair after surgical injury is unknown. Previous observations indicated seeding of denuded mesothelium by free-floating mesothelial cells may contribute to mesothelial healing. In this study, we investigated the prevalence of mesothelial cells in pleural fluid during the 7 days following pulmonary surgery...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29669318/neurod-expression-in-podocytes-and-interrelationships-with-nephrin-at-both-nuclear-and-cytoplasmic-sites
#17
Silvia Armelloni, Masami Ikehata, Deborah Mattinzoli, Min Li, Carlo Maria Alfieri, Mariapia Rastaldi, Piergiorgio Messa
Background/Aims The research of genes implicated in kidney glomerular function, eliciting cell fate program, is always at the forefront in nephrological studies. Several neurological molecules have been recently the object of study not only for their involvement in the central nervous system differentiation but also for their importance in the functionality of other organs and for mature phenotype, as in kidney. NeuroD, in CNS, is related to two functional roles, the early survival and the differentiation. The aim of our study was to ascertain the presence of NeuroD transcription factor in glomeruli and to understand which targets and mechanisms NeuroD controls...
April 13, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29663071/cyclosporine-a-responsive-congenital-nephrotic-syndrome-with-single-heterozygous-variants-in-nphs1-nphs2-and-plce1
#18
Anna Eichinger, Sabine Ponsel, Carsten Bergmann, Roman Günthner, Julia Hoefele, Kerstin Amann, Bärbel Lange-Sperandio
BACKGROUND: Congenital nephrotic syndrome (CNS) is primarily a monogenetic disease, with the majority of cases due to changes in five different genes: the nephrin (NPHS1), podocin (NPHS2), Wilms tumor 1 (WT1), laminin ß2 (LAMB2), and phospholipase C epsilon 1 (PLCE1, NPHS3) gene. Usually CNS is not responsive to immunosuppressive therapy, but treatment with ACE inhibitors, AT1 receptor blockade and/or indomethacin can reduce proteinuria. If the disease progresses to end-stage renal disease, kidney transplantation is the therapy of choice...
April 16, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29662637/the-wilms-tumor-gene-1-is-a-prognostic-factor-in-myelodysplastic-syndrome-a-meta-analysis
#19
Yanan Jiang, Lin Liu, Jinhuan Wang, Zeng Cao, Zhigang Zhao
Previous studies have suggested that Wilms' tumor gene-1 (WT1) may be related to a decrease in both relapse-free survival (RFS) and overall survival (OS) for patients with myelodysplastic syndrome (MDS). Therefore, we conducted a meta-analysis on the utility of WT1 as a prognostic indicator of MDS. Published reports were searched in the following databases: Cochrane Library, PubMed, Embase, and Web of Science. The meta-analysis was conducted using the Cochrane Collaboration RevMan 5.2 software. Six publications with 450 total patients met the inclusion criteria and were subjected to further examination...
March 23, 2018: Oncotarget
https://www.readbyqxmd.com/read/29662608/wt1-p53-and-p16-expression-in-the-diagnosis-of-low-and-high-grade-serous-ovarian-carcinomas-and-their-relation-to-prognosis
#20
Luis Felipe Sallum, Liliana Andrade, Susana Ramalho, Amanda Canato Ferracini, Rodrigo de Andrade Natal, Angelo Borsarelli Carvalho Brito, Luis Otávio Sarian, Sophie Derchain
Objective: To evaluate the diagnostic and prognostic value of the immunohistochemical expression of WT1, p53 and p16 in low- (LGSOCs) and high-grade serous ovarian carcinomas (HGSOCs). Results: HGSOC had a significantly higher proportion of advanced stage disease, higher CA125 levels, higher proportion of post-surgery residual disease and higher recurrence or disease progression. WT1 was expressed in 71.4% of LGSOCs and in 57.1% of HGSOCs ( p = 0.32). Focal and/or complete absence of p53 expression with negative p16 expression was found in 90...
March 23, 2018: Oncotarget
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