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https://www.readbyqxmd.com/read/29775646/the-rna-binding-protein-ars2-supports-hematopoiesis-at-multiple-levels
#1
Seerat Elahi, Shawn M Egan, G Aaron Holling, Rachel L Kandefer, Michael J Nemeth, Scott H Olejniczak
Recent biochemical characterization of Arsenic resistance protein 2 (Ars2) has established it as central to determining the fate of nascent RNA polymerase II (RNAPII) transcripts. Through interactions with the nuclear 5'-7-methylguanosine (7mG) cap binding complex (CBC), Ars2 promotes co-transcriptional processing coupled with nuclear export or degradation of several classes of RNAPII transcripts, allowing for gene expression programs that facilitate rapid and sustained proliferation of immortalized cells in culture...
May 15, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29775645/mesenchymal-stromal-cells-induce-a-permissive-state-in-the-bone-marrow-that-enhances-g-csf-induced-hematopoietic-stem-cell-mobilization-in-mice
#2
Evert-Jan F M de Kruijf, Rob Zuijderduijn, Marjolein C Stip, Willem E Fibbe, Melissa van Pel
Mesenchymal stromal cells (MSC) support hematopoietic stem cells (HSC) in vivo and enhance HSC engraftment and hematopoietic recovery upon co-transplantation with HSC. These data have led to the hypothesis that MSC may impact the HSC niche, leading to changes in HSC retention and trafficking. We studied the effect of MSC administration on the HSC compartment in the bone marrow (BM) in mice. Following injection of MSC, HSC numbers in the BM were decreased coinciding with an increased cell cycle activity compared to PBS-injected controls...
May 15, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29775636/epithelial-proliferation-in-inflammatory-skin-disease-is-regulated-by-ttc7-in-fibroblasts-and-lymphocytes
#3
Ursina Nüesch, Andrea A Mauracher, Lennart Opitz, Benjamin Volkmer, Katarzyna Michalak-Mićka, Jivko Kamarachev, Tom Hartwig, Ernst Reichmann, Burkhard Becher, Stefano Vavassori, Jana Pachlopnik Schmid
BACKGROUND: Mutations in tetratricopeptide repeat domain 7A (TTC7A) and its mouse orthologue Ttc7 result in a multisystemic disease, mostly affecting epithelial barriers and the immune system. Despite successful hematopoietic stem cell transplantation, ongoing progression of gastrointestinal manifestations can be life-threatening in TTC7A-deficient patients. OBJECTIVE: To identify whether TTC7A mutations dysregulate epithelial cells only, or whether a cell-intrinsic defect in lymphocytes or other cells contributes to disease manifestations...
May 15, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29775011/-hyper-ige-syndromes
#4
REVIEW
Y Y He, B Liu, X P Xiao
A hyper-IgE syndrome is a rare immunodeficiesncy disease, often accompanied by high serum IgE. It often characterized by facial features, repeated skin infections, eczema and pulmonary infection, including autosomal dominant HIES (AD-HIES) and autosomal recessive HIES (AR-HIES). AR-HIES is caused by mutations in STAT3, which is presented as connective tissue, bone, vascular disease, and high brain white matter signal. AD-HIES is mainly caused by mutations in DOCK8 and TYK2, which is presented as severe viral infection and poor prognosis...
June 5, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29774169/phase-2-study-of-everolimus-for-relapsed-or-refractory-classical-hodgkin-lymphoma
#5
Patrick B Johnston, Lauren C Pinter-Brown, Ghulam Warsi, Kristen White, Radhakrishnan Ramchandren
Background: The current standard of care for classical Hodgkin lymphoma (HL) is multiagent chemotherapy with or without radiation. In patients who relapse or fail to respond, additional high-dose chemotherapy with autologous hematopoietic stem cell transplantation (AHSCT) can improve progression-free survival (PFS). Novel therapies are required for patients refractory to chemotherapy and AHSCT. The mammalian target of rapamycin inhibitor everolimus has shown preliminary activity in preclinical models of HL and promising efficacy in patients with relapsed or refractory HL...
2018: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/29774106/hematopoietic-restricted-ptpn11e76k-reveals-indolent-mpn-progression-in-mice
#6
Stefan P Tarnawsky, Wen-Mei Yu, Cheng-Kui Qu, Rebecca J Chan, Mervin C Yoder
Juvenile Myelomonocytic Leukemia (JMML) is a pediatric myeloproliferative neoplasm (MPN) that has a poor prognosis. Somatic mutations in Ptpn11 are the most frequent cause of JMML and they commonly occur in utero . Animal models of mutant Ptpn11 have probed the signaling pathways that contribute to JMML. However, existing models may inappropriately exacerbate MPN features by relying on non-hematopoietic-restricted Cre-loxP strains or transplantations into irradiated recipients. In this study we generate hematopoietic-restricted models of Ptpn11E76K-mediated disease using Csf1r-MCM and Flt3Cre...
April 24, 2018: Oncotarget
https://www.readbyqxmd.com/read/29773641/il1rap-potentiates-multiple-oncogenic-signaling-pathways-in-aml
#7
Kelly Mitchell, Laura Barreyro, Tihomira I Todorova, Samuel J Taylor, Iléana Antony-Debré, Swathi-Rao Narayanagari, Luis A Carvajal, Joana Leite, Zubair Piperdi, Gopichand Pendurti, Ioannis Mantzaris, Elisabeth Paietta, Amit Verma, Kira Gritsman, Ulrich Steidl
The surface molecule interleukin-1 receptor accessory protein (IL1RAP) is consistently overexpressed across multiple genetic subtypes of acute myeloid leukemia (AML) and other myeloid malignancies, including at the stem cell level, and is emerging as a novel therapeutic target. However, the cell-intrinsic functions of IL1RAP in AML cells are largely unknown. Here, we show that targeting of IL1RAP via RNA interference, genetic deletion, or antibodies inhibits AML pathogenesis in vitro and in vivo, without perturbing healthy hematopoietic function or viability...
May 17, 2018: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29773600/the-interleukin-3-receptor-cd123-targeted-sl-401-mediates-potent-cytotoxic-activity-against-cd34-cd123-cells-from-acute-myeloid-leukemia-myelodysplastic-syndrome-patients-and-healthy-donors
#8
Rajeswaran Mani, Swagata Goswami, Bhavani Gopalakrishnan, Rahul Ramaswamy, Ronni Wasmuth, Minh Tranh, Xiaokui Mo, Amber Gordon, Donna Bucci, David M Lucas, Alice Mims, Christopher Brooks, Adrienne Dorrance, Alison Walker, William Blum, John C Byrd, Gerard Lozanski, Sumithira Vasu, Natarajan Muthusamy
Diseases with clonal hematopoiesis such as myelodysplastic syndrome and acute myeloid leukemia have high rates of relapse. Only a small subset of acute myeloid leukemia patients are cured with chemotherapy alone. Relapse in these diseases occurs at least in part due to the failure to eradicate leukemic stem cells or hematopoietic stem cells in myelodysplastic syndrome. CD123, the alpha chain of the interleukin-3 receptor heterodimer, is expressed on the majority of leukemic stem cells and myelodysplastic syndrome hematopoietic stem cells and in 80% of acute myeloid leukemia...
May 17, 2018: Haematologica
https://www.readbyqxmd.com/read/29773597/hematopoietic-stem-cell-loss-and-hematopoietic-failure-in-severe-aplastic-anemia-is-driven-by-macrophages-and-aberrant-podoplanin-expression
#9
Amanda McCabe, Julianne N P Smith, Angelica Costello, Jackson Maloney, Divya Katikaneni, Katherine C MacNamara
Severe aplastic anemia results from profound hematopoietic stem cell loss. T cells and interferon gamma have long been associated with severe aplastic anemia, yet the underlying mechanisms driving hematopoietic stem cell loss remain unknown. Using a mouse model of severe aplastic anemia, we demonstrate that interferon gamma-dependent hematopoietic stem cell loss required macrophages. Interferon gamma was necessary for bone marrow macrophage persistence, despite loss of other myeloid cells and hematopoietic stem cells...
May 17, 2018: Haematologica
https://www.readbyqxmd.com/read/29773496/reflections-on-methodical-approaches-to-hematopoietic-stem-cell-collection-in-children
#10
REVIEW
Claudia Del Fante, Jerard Seghatchian, Cesare Perotti
Pediatric peripheral blood stem cell collection (PBSC) is challenging because it has potentially more side effects than in adults due to the small body mass and unique physiology of children. The extracorporeal volume of the cell separator device, poor venous access and metabolic complications due to citrate toxicity are the main problems to face during PBSC collection. These aspects are more relevant in very low body weight (BW) children of 20 kg or lower. An efficient, experienced and well-prepared team of pediatricians, apheresis physicians and nurses, and physicians involved in CVC positioning is crucial to performing a safe PBSC collection...
May 9, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29773298/veno-venous-extracorporeal-membrane-oxygenation-bridged-living-donor-lung-transplantation-for-rapid-progressive-respiratory-failure-with-pleuroparenchymal-fibroelastosis-after-allogeneic-hematopoietic-stem-cell-transplantation
#11
Ayako Shimada, Jiro Terada, Kenji Tsushima, Yoshihisa Tateishi, Ryuzo Abe, Shigeto Oda, Motomu Kobayashi, Masaomi Yamane, Takahiro Oto, Koichiro Tatsumi
Cases of extracorporeal membrane oxygenation (ECMO) bridged lung transplantation (LTx) are rare in Japan because an allocation system to prioritize patients based on urgency remains to be established. For critically ill patients who cannot wait for a brain-dead donor LTx, ECMO bridge to living-donor LTx may be the only practical option. A 21-year-old woman with pleuroparenchymal fibroelastosis after hematopoietic stem cell transplantation was admitted to our hospital with rapidly progressive respiratory failure...
May 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29772816/alpha-mannosidosis-therapeutic-strategies
#12
REVIEW
Maria Rachele Ceccarini, Michela Codini, Carmela Conte, Federica Patria, Samuela Cataldi, Matteo Bertelli, Elisabetta Albi, Tommaso Beccari
Alpha-mannosidosis (α-mannosidosis) is a rare lysosomal storage disorder with an autosomal recessive inheritance caused by mutations in the gene encoding for the lysosomal α-d-mannosidase. So far, 155 variants from 191 patients have been identified and in part characterized at the biochemical level. Similarly to other lysosomal storage diseases, there is no relationship between genotype and phenotype in alpha-mannosidosis. Enzyme replacement therapy is at the moment the most effective therapy for lysosomal storage disease, including alpha-mannosidosis...
May 17, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29772764/the-making-of-leukemia
#13
REVIEW
Inés González-Herrero, Guillermo Rodríguez-Hernández, Andrea Luengas-Martínez, Marta Isidro-Hernández, Rafael Jiménez, Maria Begoña García-Cenador, Francisco Javier García-Criado, Isidro Sánchez-García, Carolina Vicente-Dueñas
Due to the clonal nature of human leukemia evolution, all leukemic cells carry the same leukemia-initiating genetic lesions, independently of the intrinsic tumoral cellular heterogeneity. However, the latest findings have shown that the mode of action of oncogenes is not homogeneous throughout the developmental history of leukemia. Studies on different types of hematopoietic tumors have shown that the contribution of oncogenes to leukemia is mainly mediated through the epigenetic reprogramming of the leukemia-initiating target cell...
May 17, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29772541/myeloid-heme-oxygenase-1-a-new-therapeutic-target-in-anti-inflammation
#14
Mingyi Zhao, Minghua Yang, Weitao Que, Lin Zhong, Masayuki Fujino, Xiao-Kang Li
An increasing amount of evidence reveals that an orchestrated interplay between myeloid subpopulations in the hematopoietic system plays a significant role in supporting normal functions of the immune system and facilitating homeostatic restoration upon exogenous or endogenous insults. Heme oxygenase-1 (HO-1), a microsomal enzyme discovered decades ago, can metabolize pro-oxidant heme into biliverdin, free iron, and carbon monoxide. This enzymatic reaction produces biological materials, contributing to major immunomodulatory effects...
June 1, 2018: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/29772351/a-couple-based-communication-intervention-for-hematopoietic-cell-transplant-survivors-and-their-caregiving-partners-feasibility-acceptability-and-change-in-process-measures
#15
Shelby Langer, Laura Porter, Joan Romano, Michael Todd, Stephanie Lee
Hematopoietic cell transplantation (HCT) poses significant challenges for recipients and their caregiving partners. Couples may refrain from talking about treatment-related fears and concerns in order to minimize distress. This single-group, pre-post study examined feasibility and acceptability of an intervention designed to optimize communication between HCT patients and partners; it also assessed change in process measures. Couples met with a therapist five times to learn skills for disclosing illness-related thoughts and feelings and responding supportively to one another...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29772350/unmanipulated-haploidentical-hematopoietic-stem-cell-transplantation-achieved-outcomes-comparable-with-matched-unrelated-donor-transplantation-in-young-acquired-severe-aplastic-anemia
#16
Yue Lu, Rui-Juan Sun, Yan-Li Zhao, Min Xiong, Xing-Yu Cao, Jing-Pin Zhang, Zhi-Jie Wei, Jia-Rui Zhou, De-Yan Liu, Dao-Pei Lu
Salvage haploidentical HSCT is considered in patients with severe aplastic anemia (SAA) if matched unrelated donor (MUD) is unavailable. However, studies on haplo- and MUD transplantation in SAA are yet lacking. The present study retrospectively analyzed the outcomes of 89 young SAA patients who underwent unmanipulated alternative hematopoietic stem cell transplantation (HSCT) between September 2012 and September 2016 at our single center. 41 patients received haploidentical donors, and 48 patients received MUD for HSCT...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29772232/hypomyelination-and-cognitive-impairment-in-mice-lacking-cd133-prominin-1
#17
Mi-Hyun Choi, Ji Eun Na, Ye Ran Yoon, Im Joo Rhyu, Young-Gyu Ko, Ja-Hyun Baik
The CD133 antigen, also known as prominin-1, is a glycoprotein that specifically localizes to plasma membrane protrusions. The precise function of CD133 remains unknown, but it is expressed in various progenitor cells including those derived from the neural and hematopoietic system, as well as different tissues. In the adult mouse brain, CD133 is highly expressed in white matter. Here, we performed immunochemical staining and electron microscopy to demonstrate that mice lacking CD133 (CD133-/- ) exhibit decreased myelin in the corpus callosum, the largest white matter tract in the brain...
May 14, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29771974/correction-characteristics-and-risk-of-chronic-graft-versus-host-disease-of-liver-in-allogeneic-hematopoietic-stem-cell-transplant-recipients
#18
Chien-Ting Chen, Chun-Yu Liu, Yuan-Bin Yu, Chia-Jen Liu, Liang-Tsai Hsiao, Jyh-Pyng Gau, Tzeon-Jye Chiou, Jing-Hwang Liu, Yao-Chung Liu
[This corrects the article DOI: 10.1371/journal.pone.0185210.].
2018: PloS One
https://www.readbyqxmd.com/read/29771962/progression-of-the-radiologic-severity-index-predicts-mortality-in-patients-with-parainfluenza-virus-associated-lower-respiratory-infections
#19
Ajay Sheshadri, Dimpy P Shah, Myrna Godoy, Jeremy J Erasmus, Juhee Song, Liang Li, Scott E Evans, Roy F Chemaly, Burton F Dickey, David E Ost
BACKGROUND: Radiologic severity may predict adverse outcomes after lower respiratory tract infection (LRI). However, few studies have quantified radiologic severity of LRIs. We sought to evaluate whether a semi-quantitative scoring tool, the Radiologic Severity Index (RSI), predicted mortality after parainfluenza virus (PIV)-associated LRI. METHODS: We conducted a retrospective review of consecutively-enrolled adult patients with hematologic malignancy or hematopoietic stem cell transplantation and with PIV detected in nasal wash who subsequently developed radiologically-confirmed LRI...
2018: PloS One
https://www.readbyqxmd.com/read/29771921/mosquito-saliva-alone-has-profound-effects-on-the-human-immune-system
#20
Megan B Vogt, Anismrita Lahon, Ravi P Arya, Alexander R Kneubehl, Jennifer L Spencer Clinton, Silke Paust, Rebecca Rico-Hesse
Mosquito saliva is a very complex concoction of >100 proteins, many of which have unknown functions. The effects of mosquito saliva proteins injected into our skin during blood feeding have been studied mainly in mouse models of injection or biting, with many of these systems producing results that may not be relevant to human disease. Here, we describe the numerous effects that mosquito bites have on human immune cells in mice engrafted with human hematopoietic stem cells. We used flow cytometry and multiplex cytokine bead array assays, with detailed statistical analyses, to detect small but significant variations in immune cell functions after 4 mosquitoes fed on humanized mice footpads...
May 2018: PLoS Neglected Tropical Diseases
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