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https://www.readbyqxmd.com/read/28811308/wilms-tumour-1-wt1-in-development-homeostasis-and-disease
#1
REVIEW
Nicholas D Hastie
The study of genes mutated in human disease often leads to new insights into biology as well as disease mechanisms. One such gene is Wilms' tumour 1 (WT1), which plays multiple roles in development, tissue homeostasis and disease. In this Primer, I summarise how this multifaceted gene functions in various mammalian tissues and organs, including the kidney, gonads, heart and nervous system. This is followed by a discussion of our current understanding of the molecular mechanisms by which WT1 and its two major isoforms regulate these processes at the transcriptional and post-transcriptional levels...
August 15, 2017: Development
https://www.readbyqxmd.com/read/28810922/pleural-epithelioid-angiosarcoma-with-lymphatic-differentiation-arisen-after-radiometabolic-therapy-for-thyroid-carcinoma-immunohistochemical-findings-and-review-of-the-literature
#2
REVIEW
Daniela Cabibi, Giulia Pipitone, Rossana Porcasi, Sabrina Ingrao, Ignazio Benza, Calogero Porrello, Massimo Cajozzo, Antonino Giulio Giannone
BACKGROUND: Pleural angiosarcoma is a rare tumor that causes diffuse pleural thickening and effusion, mimicking mesothelioma. Immunohistochemistry is needed to highlight endothelial differentiation. We describe the first case of pleural angiosarcoma with lymphatic differentiation following radiometabolic therapy for thyroid carcinoma. CASE PRESENTATION: A 50-year-old man showed diffuse pleural thickening and effusion. Nine years earlier, he underwent thyroidectomy and radiometabolic therapy for thyroid carcinoma with lymph node metastases...
August 15, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28810324/-minimal-residual-disease-level-predicts-outcomes-in-the-non-favorable-risk-patients-with-acute-myeloid-leukemia
#3
X Ren, T Zhao, J Wang, H H Zhu, H Jiang, J S Jia, S M Yang, B Jiang, D B Wang, X J Huang, Q Jiang
Objective: To explore impact of minimal residual leukemia (MRD) on outcomes in the non-favorable risk adults with de novo acute myeloid leukemia (AML) . Methods: From January 2008 to February 2016, data of consecutive newly-diagnosed non-favorable risk adults with AML (non-APL) according to SWOG criteria who achieved morphologic leukemia-free state (MLFS) and received continuous chemotherapy were assessed retrospectively. Results: 292 AML patients were enrolled, 150 (51.4%) were male. Median age was 46 years (range, 18-65 years) ...
July 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28810297/-clinical-and-pathologic-features-of-extrapleural-sarcomatoid-mesothelioma
#4
M C Wei, S J Yang
Objective: To investigate the morphological features, diagnosis and differential diagnosis of extrapleural sarcomatoid malignant mesothelioma (SMM). Methods: Six cases of extrapleural SMM were evaluated for their clinical, histological, immunohistochemical features, and prognosis. Results: Patients included 3 men and 3 women, with a median age of 60 years (range 41-75 years). All patients had no asbestos exposure in history and no pleural lesions. The tumors involved peritoneum (3 cases), bone (2 cases), and neck soft tissue (1 case)...
August 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28809397/fibroblasts-in-an-endocardial-fibroelastosis-disease-model-mainly-originate-from-mesenchymal-derivatives-of-epicardium
#5
Hui Zhang, Xiuzhen Huang, Kuo Liu, Juan Tang, Lingjuan He, Wenjuan Pu, Qiaozhen Liu, Yan Li, Xueying Tian, Yue Wang, Libo Zhang, Ying Yu, Hongyan Wang, Ronggui Hu, Fengchao Wang, Ting Chen, Qing-Dong Wang, Zengyong Qiao, Li Zhang, Kathy O Lui, Bin Zhou
Endocardial fibroelastosis (EFE) refers to the thickening of the ventricular endocardium as a result of de novo deposition of subendocardial fibrous tissue layers during neonatal heart development. The origin of EFE fibroblasts is proposed to be postnatal endocardial cells that undergo an aberrant endothelial-to-mesenchymal transition (EndMT). Genetic lineage tracing of endocardial cells with the inducible endocardial Cre line Npr3-CreER and the endothelial cell tracing line Cdh5-CreER on an EFE-like model did not reveal any contribution of neonatal endocardial cells to fibroblasts in the EFE-like tissues...
August 15, 2017: Cell Research
https://www.readbyqxmd.com/read/28801348/incidental-detection-of-germline-variants-of-potential-clinical-significance-by-massively-parallel-sequencing-in-haematological-malignancies
#6
Costas K Yannakou, Kate Jones, Georgina L Ryland, Ella R Thompson, Gareth Reid, Michelle McBean, Alison Trainer, David Westerman, Piers Blombery
Massively parallel sequencing (MPS) technology has become routinely available for diagnosis, prognostication and therapeutic decision-making in haematological malignancies. However, increased throughput and wider coverage of genes can have unintended consequences. Germline variants of potential clinical significance (GVPCSs) detected during cancer testing may have implications for patients and families beyond the biological evaluation of a specific tumour. 721 reports generated from MPS panels used in the routine testing of myeloid and lymphoid malignancies were reviewed and variants within genes of potential germline relevance (TP53, RUNX1, GATA2 and WT1 in all contexts and CBL, KRAS and NRAS in the setting of juvenile myelomonocytic leukaemia) were analysed...
August 11, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28795841/serous-carcinoma-mimicking-primary-urothelial-carcinoma-on-clinical-evaluation-and-pathology-a-potential-diagnostic-pitfall
#7
Leili Mirsadraei, Alexey Hodkoff, Karra Jones, Ahmed Shabaik, A Karim Kader, Cheryl C Saenz, Rodolfo Montironi, David E Tacha, Oluwole Fadare, Donna E Hansel
CONTEXT: - Serous carcinoma of the gynecologic tract often involves the external bladder wall and can occasionally mimic primary urothelial carcinoma of the bladder. OBJECTIVE: - To define the spectrum of morphologic and immunohistochemical features that characterize serous carcinoma involving the bladder wall and its distinction from urothelial carcinoma. DESIGN: - We reviewed all cases of serous carcinoma secondarily involving the bladder wall from the University of California San Diego and Polytechnic Institute for histopathologic and immunohistochemical features...
August 10, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28794400/tbx18-positive-cells-differentiated-from-murine-es-cells-serve-as-proepicardial-progenitors-to-give-rise-to-vascular-smooth-muscle-cells-and-fibroblasts
#8
Nobuhito Ikeda, Natsumi Nakazawa, Yasutaka Kurata, Hisako Yaura, Fikri Taufiq, Hiroyuki Minato, Akio Yoshida, Haruaki Ninomiya, Yuji Nakayama, Masanari Kuwabara, Yasuaki Shirayoshi, Ichiro Hisatome
Proepicardium (PE) cells generate cardiac fibroblasts, smooth muscle cells (SMCs) and endothelial cells that form coronary arteries. T-box18 (Tbx18) is a well-known marker of PE cells and epicardium. We examined whether Tbx18-positive cells differentiated from murine embryonic stem (ES) cells serve as PE progenitors to give rise to vascular SMCs and fibroblasts. To collect Tbx18-positive cells, we established Tbx18-EGFP knock-in mouse ES cells using the CRISPR/Cas9 system. We harvested the Tbx18-EGFP-positive cells on day 8, 10 and 14 after the initiation of differentiation; Tbx18 mRNA was enriched on day 8 to 14 and Snai2 mRNA was enriched on day 8 and 10, indicating successful collection of Tbx18-positive cells...
2017: Biomedical Research
https://www.readbyqxmd.com/read/28794289/differentiation-of-mesenchymal-stem-cells-towards-nephrogenic-lineage-and-their-enhanced-resistance-to-oxygen-peroxide-induced-oxidative-stress
#9
Asima Tayyeb, Naveed Shahzad, Gibran Ali
INTRODUCTION: Mesenchymal stem cells (MSCs) have been publicized to ameliorate kidney injury both in vitro and in vivo. However, very less is known if MSCs can be differentiated towards renal lineages and their further application potential in kidney injuries. MATERIALS AND METHODS: The present study developed a conditioning system of growth factors fibroblast growth factor 2, transforming growth factor-β2, and leukemia inhibitory factor for in vitro differentiation of MSCs isolated from different sources towards nephrogenic lineage...
July 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28781196/clinicopathological-and-immunohistochemical-analysis-of-epithelial-lined-true-cysts-of-the-adrenal-gland-with-proposal-of-a-new-histogenetic-categorization
#10
Łukasz Koperski, Paweł Pihowicz, Benedykt Szczepankiewicz, Łukasz Fus, Agata Cyran, Magdalena Bogdańska, Barbara Górnicka
Epithelial- lined (true) cysts are rare lesions and until now the only information we had about their histogenesis was based on the analysis of a few cases. We retrospectively reviewed 8 cases of cysts with a true epithelial lining (confirmed immunohistochemically). The pathological findings and immunohistochemical analysis of the epithelial linings allowed for categorization of the cysts into 3 groups. Five cysts had pure mesothelial lining, which was flattened to cuboidal, and demonstrated a positive reaction for mesothelial markers (eg...
July 21, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28776572/molecular-based-classification-algorithm-for-endometrial-carcinoma-categorizes-ovarian-endometrioid-carcinoma-into-prognostically-significant-groups
#11
Carlos Parra-Herran, Jordan Lerner-Ellis, Bin Xu, Sam Khalouei, Dina Bassiouny, Matthew Cesari, Nadia Ismiil, Sharon Nofech-Mozes
The Cancer Genome Atlas classification divides endometrial carcinoma in biologically distinct groups, and testing for p53, mismatch repair proteins (MMR), and polymerase ɛ (POLE) exonuclease domain mutations has been shown to predict the molecular subgroup and clinical outcome. While abnormalities in these markers have been described in ovarian endometrioid carcinoma, their role in predicting its molecular profile and prognosis is still not fully explored. Patients with ovarian endometrioid carcinomas treated surgically in a 14-year period were selected...
August 4, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28771903/fludarabine-cytarabine-g-csf-and-idarubicin-for-children-with-relapsed-aml
#12
Hideki Nakayama, Daisuke Tomizawa, Shiro Tanaka, Shotaro Iwamoto, Akira Shimada, Akiko M Saito, Yuka Yamashita, Hiroshi Moritake, Kiminori Terui, Takashi Taga, Hidemasa Matsuo, Yoshiyuki Kosaka, Katsuyoshi Koh, Hajime Hosoi, Hidemitsu Kurosawa, Keiichi Isoyama, Keizo Horibe, Shuki Mizutani, Souichi Adachi
BACKGROUND: The combination of fludarabine (Flu), high dose cytarabine (Ara-C) and granulocyte-colony stimulating factor (G-CSF) called "FLAG" with anthracyclines has become a standard chemotherapy for refractory acute myeloid leukemia (AML) in European children and adults. To clarify the efficacy and the safety of FLAG-idarubicin (IDA) for children prospectively, we planned a multicenter phase II study (AML-R11) by Japanese Pediatric Leukemia/Lymphoma Study Group. METHODS: Patients with AML at the age between 2 and 20 years old, who had the first bone marrow (BM) relapse or induction failure, were enrolled...
August 3, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28768736/congenital-diaphragmatic-hernias-from-genes-to-mechanisms-to-therapies
#13
REVIEW
Gabrielle Kardon, Kate G Ackerman, David J McCulley, Yufeng Shen, Julia Wynn, Linshan Shang, Eric Bogenschutz, Xin Sun, Wendy K Chung
Congenital diaphragmatic hernias (CDHs) and structural anomalies of the diaphragm are a common class of congenital birth defects that are associated with significant morbidity and mortality due to associated pulmonary hypoplasia, pulmonary hypertension and heart failure. In ∼30% of CDH patients, genomic analyses have identified a range of genetic defects, including chromosomal anomalies, copy number variants and sequence variants. The affected genes identified in CDH patients include transcription factors, such as GATA4, ZFPM2, NR2F2 and WT1, and signaling pathway components, including members of the retinoic acid pathway...
August 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28765625/blood-testis-barrier-integrity-depends-on-pin1-expression-in-sertoli-cells
#14
Rabia Islam, Heein Yoon, Bong-Soo Kim, Han-Sol Bae, Hye-Rim Shin, Woo-Jin Kim, Won-Joon Yoon, Yun-Sil Lee, Kyung Mi Woo, Jeong-Hwa Baek, Hyun-Mo Ryoo
The conformation and function of a subset of serine and threonine-phosphorylated proteins are regulated by the prolyl isomerase Pin1 through isomerization of phosphorylated Ser/Thr-Pro bonds. Pin1 is intensely expressed in Sertoli cells, but its function in this post mitotic cell remains unclear. Our aim was to investigate the role of Pin1 in the Sertoli cells. Lack of Pin1 caused disruption of the blood-testis barrier. We next investigated if the activin pathways in the Sertoli cells were affected by lack of Pin1 through immunostaining for Smad3 protein in testis tissue...
August 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28748349/desmoplastic-small-round-cell-tumor-evaluation-of-reverse-transcription-polymerase-chain-reaction-and-fluorescence-in-situ-hybridization-as-ancillary-molecular-diagnostic-techniques
#15
Mustafa Mohamed, David Gonzalez, Karen J Fritchie, John Swansbury, Dorte Wren, Charlotte Benson, Robin L Jones, Cyril Fisher, Khin Thway
Desmoplastic small round cell tumor (DSRCT) is a rare, biologically aggressive soft tissue neoplasm of uncertain differentiation, most often arising in the abdominal and pelvic cavities of adolescents and young adults with a striking male predominance. Histologically, it is characterized by islands of uniform small round cells in prominent desmoplastic stroma, and it has a polyimmunophenotypic profile, typically expressing WT1 and cytokeratin, desmin, and neural/neuroendocrine differentiation markers to varying degrees...
July 26, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28739660/a-gene-implicated-in-activation-of-retinoic-acid-receptor-targets-is-a-novel-renal-agenesis-gene-in-humans
#16
Patrick D Brophy, Maria Rasmussen, Mrutyunjaya Parida, Greg Bonde, Benjamin W Darbro, Xiaojing Hong, Jason C Clarke, Kevin A Peterson, James Denegre, Michael Schneider, Caroline R Sussman, Lone Sunde, Dorte L Lildballe, Jens Michael Hertz, Robert A Cornell, Stephen A Murray, John R Manak
Renal agenesis (RA) is one of the more extreme examples of congenital anomalies of the kidney and urinary tract (CAKUT). Bilateral renal agenesis is almost invariably fatal at birth, and unilateral renal agenesis can lead to future health issues including end stage renal disease. Genetic investigations have identified several gene variants which cause RA, including EYA1, LHX1, and WT1 However, whereas compound null mutations of genes encoding α and γ retinoic acid receptors (RARs) cause RA in mice, to date there have been no reports of variants in RAR genes causing RA in humans...
July 24, 2017: Genetics
https://www.readbyqxmd.com/read/28737171/brg1-swi-snf-dependent-regulation-of-the-wt1-transcriptional-landscape-mediates-epicardial-activity-during-heart-development-and-disease
#17
Joaquim Miguel Vieira, Sara Howard, Cristina Villa Del Campo, Sveva Bollini, Karina N Dubé, Megan Masters, Damien N Barnette, Mala Rohling, Xin Sun, Laura E Hankins, Daria Gavriouchkina, Ruth Williams, Daniel Metzger, Pierre Chambon, Tatjana Sauka-Spengler, Benjamin Davies, Paul R Riley
Epicardium-derived cells (EPDCs) contribute cardiovascular cell types during development and in adulthood respond to Thymosin β4 (Tβ4) and myocardial infarction (MI) by reactivating a fetal gene programme to promote neovascularization and cardiomyogenesis. The mechanism for epicardial gene (re-)activation remains elusive. Here we reveal that BRG1, the essential ATPase subunit of the SWI/SNF chromatin-remodelling complex, is required for expression of Wilms' tumour 1 (Wt1), fetal EPDC activation and subsequent differentiation into coronary smooth muscle, and restores Wt1 activity upon MI...
July 24, 2017: Nature Communications
https://www.readbyqxmd.com/read/28736882/the-clinical-significance-of-monitoring-the-expression-of-the-sil-tal1-fusion-gene-in-t-cell-acute-lymphoblastic-leukemia-after-allogeneic-hematopoietic-stem-cell-transplantation
#18
X Zhao, Y Hong, Y Qin, Y Xu, Y Chang, Y Wang, X Zhang, L Xu, X Huang
INTRODUCTION: SIL-TAL1 rearrangement is common in T-cell acute lymphoblastic leukemia (T-ALL). However, whether this fusion gene might be used as a reliable marker of minimal residual disease (MRD) following allogeneic stem cell transplantation (allo-HSCT) remains unknown METHODS: The clinical data of consecutive 29 patients with T-ALL who received allo-HSCT were collected. Their MRD were evaluated by SIL-TAL1, Wilms' tumor 1 (WT1) expression, and the leukemia-associated immunophenotype (LAIP) ...
July 24, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28728504/the-diverse-expression-of-the-wt1-gene-in-patients-with-acquired-bone-marrow-failure-syndromes
#19
Yahong You, Jiali Huo, Shihong Lu, Yingqi Shao, Meili Ge, Jun Shi, Xingxin Li, Jinbo Huang, Zhendong Huang, Jing Zhang, Min Wang, Neng Nie, Yizhou Zheng
Acquired bone marrow failure syndromes (aBMFS) encompass a wide range of diseases. A study to investigate WT1 expression in BM was conducted in 387 patients with aBMFS in China. The WT1 level in patients with aplastic anemia (AA) was significantly lower than that in patients with paroxysmal nocturnal hemoglobinuria (PNH, p = .023) and myelodysplastic syndrome (MDS, p < .001). In addition, the WT1 level in patients with MDS significantly increased as the disease progressed to an advanced stage. Patients with hypoplastic MDS had a differentiated expression level of WT1 compared with that of NSAA (p < ...
July 21, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28724775/n-degradomic-analysis-reveals-a-proteolytic-network-processing-the-podocyte-cytoskeleton
#20
Markus M Rinschen, Ann-Kathrin Hoppe, Florian Grahammer, Martin Kann, Linus A Völker, Eva-Maria Schurek, Julie Binz, Martin Höhne, Fatih Demir, Milena Malisic, Tobias B Huber, Christine Kurschat, Jayachandran N Kizhakkedathu, Bernhard Schermer, Pitter F Huesgen, Thomas Benzing
Regulated intracellular proteostasis, controlled in part by proteolysis, is essential in maintaining the integrity of podocytes and the glomerular filtration barrier of the kidney. We applied a novel proteomics technology that enables proteome-wide identification, mapping, and quantification of protein N-termini to comprehensively characterize cleaved podocyte proteins in the glomerulus in vivo We found evidence that defined proteolytic cleavage results in various proteoforms of important podocyte proteins, including those of podocin, nephrin, neph1, α-actinin-4, and vimentin...
July 19, 2017: Journal of the American Society of Nephrology: JASN
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