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protein S deficiency

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https://www.readbyqxmd.com/read/29350465/proteomics-analysis-of-skeletal-muscle-from-leptin-deficient-ob-ob-mice-reveals-adaptive-remodeling-of-metabolic-characteristics-and-fiber-type-composition
#1
Milena Schönke, Marie Björnholm, Alexander V Chibalin, Juleen R Zierath, Atul S Deshmukh
Skeletal muscle insulin resistance, an early metabolic defect in the pathogenesis of type 2 diabetes, may be a cause or consequence of altered protein expressions profiles. Proteomics technology offers enormous promise to investigate molecular mechanisms underlying pathologies, however, the analysis of skeletal muscle is challenging. Using state-of-the-art multi-enzyme digestion and filter-aided sample preparation (MED-FASP) and a mass spectrometry (MS) based workflow, we performed a global proteomics analysis of skeletal muscle from leptin-deficient, obese, insulin resistant (ob/ob) and lean mice in mere two fractions in a short time (8 hours/sample)...
January 19, 2018: Proteomics
https://www.readbyqxmd.com/read/29349092/cathepsin-b-plays-a-key-role-in-optimal-production-of-the-influenza-a-virus
#2
Macon D Coleman, Soon-Duck Ha, S M Mansour Haeryfar, Stephen Dominic Barr, Sung Ouk Kim
Background: Influenza A virus (IAV) is the etiologic agent of the febrile respiratory illness, commonly referred to as 'flu'. The lysosomal protease cathepsin B (CTSB) has shown to be involved in the lifecycle of various viruses. Here, we examined the role of CTSB in the IAV lifecycle. Methods: CTSB-deficient (CTSB-/-) macrophages and the human lung epithelial cell line A549 cells treated with CA-074Me were infected with the A/Puerto Rico/8/34 strain of IAV (IAV-PR8)...
2018: Journal of Virology & Antiviral Research
https://www.readbyqxmd.com/read/29349091/coexistent-ipsilateral-internal-carotid-artery-occlusion-and-cerebral-venous-thrombosis-in-hepatitis-c
#3
Karan Seegobin, Somphanh Khousakhoun, Ryan Crooks, Satish Maharaj, Cherisse Baldeo
A 58-year-old male, known to have hepatitis C virus (HCV), presented with intermittent headaches and left-sided sensorimotor symptoms. There were no focal neurological deficits on examination. Electrocardiogram was unremarkable. Computed tomography angiography head and neck displayed extracranial right internal carotid artery occlusion. Magnetic resonance imaging showed right cortical vein thrombosis, with hemorrhagic infarction. Echocardiography with bubble study was unremarkable. Hypercoagulable workup was significant for protein S deficiency...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29348879/loss-of-neil3-dna-glycosylase-markedly-increases-replication-associated-double-strand-breaks-and-enhances-sensitivity-to-atr-inhibitor-in-glioblastoma-cells
#4
Alex W Klattenhoff, Megha Thakur, Christopher S Chu, Debolina Ray, Samy L Habib, Dawit Kidane
DNA endonuclease eight-like glycosylase 3 (NEIL3) is one of the DNA glycosylases that removes oxidized DNA base lesions from single-stranded DNA (ssDNA) and non-B DNA structures. Approximately seven percent of human tumors have an altered NEIL3 gene. However, the role of NEIL3 in replication-associated repair and its impact on modulating treatment response is not known. Here, we report that NEIL3 is localized at the DNA double-strand break (DSB) sites during oxidative DNA damage and replication stress. Loss of NEIL3 significantly increased spontaneous replication-associated DSBs and recruitment of replication protein A (RPA)...
December 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29345196/surfactant-protein-c-dampens-inflammation-by-decreasing-jak-stat-activation-during-lung-repair
#5
Huiyan Jin, Andrzej K Ciechanowicz, Alanna R Kaplan, Lin Wang, Ping Xia Zhang, Yi-Chien Lu, Rachel E Tobin, Brooke A Tobin, Lauren Cohn, Caroline J Zeiss, Patty J Lee, Emanuela M Bruscia, Diane S Krause
Surfactant Protein C (SPC), a key component of pulmonary surfactant, also plays a role in regulating inflammation. SPC deficiency in patients and mouse models is associated with increased inflammation and delayed repair, but the key drivers of SPC-regulated inflammation in response to injury are largely unknown. This study focuses on a new mechanism of SPC as an anti-inflammatory molecule using SPC-TK/SPC-KO (surfactant protein C-thymidine kinase/surfactant protein C knockout) mice, which represent a novel sterile injury model that mimics clinical acute respiratory distress syndrome (ARDS)...
January 18, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29343555/antagonism-of-integrin-cd11b-affords-protection-against-endotoxin-shock-and-polymicrobial-sepsis-via-attenuation-of-hmgb1-nucleocytoplasmic-translocation-and-extracellular-release
#6
Huiting Zhou, Yanhong Li, Huan Gui, He Zhao, Ming Wu, Gang Li, Yiping Li, Zhenjiang Bai, Zhimin Yin, H Paul Redmond, Jian Wang, Jiang Huai Wang, Zhihui Zhao
High mobility group box 1 (HMGB1), a chromatin-binding nuclear protein, plays a critical role in sepsis by acting as a key "late-phase" inflammatory mediator. Integrin CD11b is essential for inflammatory cell activation and migration, thus mediating inflammatory responses. However, it is unclear whether CD11b participates in the development of sepsis. In this study, we report that CD11b contributes to LPS-induced endotoxin shock and microbial sepsis, as antagonism of CD11b with the CD11b blocking Ab or CD11b inhibitor Gu-4 protects mice against LPS- and microbial sepsis-related lethality, which is associated with significantly diminished serum HMGB1 levels...
January 17, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29341982/extensive-necrotic-skin-lesions-due-to-post-varicella-protein-s-deficiency
#7
Aymeric De Geyer, Guillaume Robert, Sylvette Marleix, Bernard Fraisse, Grégory Lucas, Pierre Tattevin, Philippe Violas
Post-varicella protein S deficiency is a rare and severe disease. We report a case of extensive necrotic skin lesions of acute onset 7 days after varicella in a 4-year old girl. Protein S antigen and activity were <10%, anti-protein S antibodies were detected. She was treated with anticoagulation, plasmapheresis, and fresh frozen plasma. She survived, but required leg amputation.
January 16, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29340929/s-adenosyl-methionine-and-transmethylation-pathways-in-neuropsychiatric-diseases-throughout-life
#8
Jin Gao, Catherine M Cahill, Xudong Huang, Joshua L Roffman, Stefania Lamon-Fava, Maurizio Fava, David Mischoulon, Jack T Rogers
S-Adenosyl methionine (SAMe), as a major methyl donor, exerts its influence on central nervous system function through cellular transmethylation pathways, including the methylation of DNA, histones, protein phosphatase 2A, and several catecholamine moieties. Based on available evidence, this review focuses on the lifelong range of severe neuropsychiatric and neurodegenerative diseases and their associated neuropathologies, which have been linked to the deficiency/load of SAMe production or/and the disturbance in transmethylation pathways...
January 16, 2018: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/29339750/a-single-dose-of-modified-vaccinia-ankara-expressing-ebola-virus-like-particles-protects-nonhuman-primates-from-lethal-ebola-virus-challenge
#9
Arban Domi, Friederike Feldmann, Rahul Basu, Nathanael McCurley, Kyle Shifflett, Jackson Emanuel, Michael S Hellerstein, Farshad Guirakhoo, Chiara Orlandi, Robin Flinko, George K Lewis, Patrick W Hanley, Heinz Feldmann, Harriet L Robinson, Andrea Marzi
Ebola virus (EBOV), isolate Makona, was the causative agent of the West African epidemic devastating predominantly Guinea, Liberia and Sierra Leone from 2013-2016. While several experimental vaccine and treatment approaches have been accelerated through human clinical trials, there is still no approved countermeasure available against this disease. Here, we report the construction and preclinical efficacy testing of a novel recombinant modified vaccinia Ankara (MVA)-based vaccine expressing the EBOV-Makona glycoprotein GP and matrix protein VP40 (MVA-EBOV)...
January 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29339402/canonical-notch-signaling-is-dispensible-for-adult-steady-state-and-stress-myelo-erythropoiesis
#10
Sara Duarte, Petter S Woll, Natalija Buza-Vidas, Desmond Wai Loon Chin, Hanane Boukarabila, Tiago C Luís, Laura Stenson, Tiphaine Bouriez-Jones, Helen Ferry, Adam J Mead, Deborah Atkinson, Shaobo Jin, Sally-Ann Clark, Bishan Wu, Emmanouela Repapi, Nicki Gray, Stephen Taylor, Anders P Mutvei, Yat Long Tsoi, Claus Nerlov, Urban Lendahl, Sten Eirik W Jacobsen
While an essential role for canonical Notch signaling in generation of hematopoietic stem cells in the embryo and in thymic T cell development is well established, its role in adult bone marrow (BM) myelopoiesis remains unclear. Some studies, analyzing myeloid progenitors in adult mice with inhibited Notch signaling, implicated distinct roles of canonical Notch signaling in regulation of progenitors for the megakaryocyte, erythroid and granulocyte-macrophage cell lineages. However, these studies might also have targeted other pathways...
January 16, 2018: Blood
https://www.readbyqxmd.com/read/29339045/deficiency-of-dietary-pyridoxine-disturbed-the-intestinal-physical-barrier-function-of-young-grass-carp-ctenopharyngodon-idella
#11
Pei Wu, Xin Zheng, Xiao-Qiu Zhou, Wei-Dan Jiang, Yang Liu, Jun Jiang, Sheng-Yao Kuang, Ling Tang, Yong-An Zhang, Lin Feng
The aim of this study was to assess the effects of dietary pyridoxine (PN) deficiency on intestinal antioxidant capacity, cell apoptosis and intercellular tight junction in young grass carp (Ctenopharyngodon idella). A total of 540 young grass carp (231.85 ± 0.63 g) were fed six diets containing graded levels of PN (0.12-7.48 mg/kg diet) for 10 weeks. At the end of the feeding trial, the fish were challenged with Aeromonas hydrophila for 2 weeks. The results showed that compared with the optimal PN level, PN deficiency (1) increased the contents of reactive oxygen species (ROS), malondialdehyde (MDA) and protein carbonyl (PC), decreased the activities and mRNA levels of antioxidant enzymes such as copper, zinc superoxide dismutase (CuZnSOD), catalase (CAT), glutathione peroxidase (GPx), glutathione-S-transferase (GST) and glutathione reductase (GR) (P < ...
January 12, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29337305/host-expression-of-pd-l1-determines-efficacy-of-pd-l1-pathway-blockade-mediated-tumor-regression
#12
Heng Lin, Shuang Wei, Elaine M Hurt, Michael D Green, Lili Zhao, Linda Vatan, Wojciech Szeliga, Ronald Herbst, Paul W Harms, Leslie A Fecher, Pankaj Vats, Arul M Chinnaiyan, Christopher D Lao, Theodore S Lawrence, Max Wicha, Junzo Hamanishi, Masaki Mandai, Ilona Kryczek, Weiping Zou
Programmed death-1 receptor (PD-L1, B7-H1) and programmed cell death protein 1 (PD-1) pathway blockade is a promising therapy for treating cancer. However, the mechanistic contribution of host and tumor PD-L1 and PD-1 signaling to the therapeutic efficacy of PD-L1 and PD-1 blockade remains elusive. Here, we evaluated 3 tumor-bearing mouse models that differ in their sensitivity to PD-L1 blockade and demonstrated a loss of therapeutic efficacy of PD-L1 blockade in immunodeficient mice and in PD-L1- and PD-1-deficient mice...
January 16, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29337245/hepatic-alcohol-dehydrogenase-deficiency-induces-pancreatic-injury-in-chronic-ethanol-feeding-model-of-deer-mice
#13
Samir M Amer, Kamlesh K Bhopale, Ramu D Kakumanu, Vsevolod L Popov, Bill A Rampy, Inas H El-Mehallawi, Magdy M Ashmawy, G A Shakeel Ansari, Bhupendra S Kaphalia
The single most common cause of chronic pancreatitis (CP, a serious inflammatory disease) is chronic alcohol abuse, which impairs hepatic alcohol dehydrogenase (ADH, a major ethanol oxidizing enzyme). Previously, we found ~5 fold greater fatty acid ethyl esters (FAEEs), and injury in the pancreas of hepatic ADH deficient (ADH-) vs. hepatic normal ADH (ADH+) deer mice fed 3.5g% ethanol via liquid diet daily for two months. Therefore, progression of ethanol-induced pancreatic injury was determined in ADH- deer mice fed ethanol for four months to delineate the mechanism and metabolic basis of alcoholic chronic pancreatitis (ACP)...
January 11, 2018: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29334734/a-method-for-selective-depletion-of-zn-ii-ions-from-complex-biological-media-and-evaluation-of-cellular-consequences-of-zn-ii-deficiency
#14
Christopher E R Richardson, Lisa S Cunden, Vincent L Butty, Elizabeth M Nolan, Stephen J Lippard, Matthew D Shoulders
We describe the preparation, evaluation, and application of an S100A12 protein-conjugated solid support, hereafter the "A12-resin," that can remove 99% of Zn(II) from complex biological solutions without significantly perturbing the concentrations of other metal ions. The A12-resin can be applied to selectively deplete Zn(II) from diverse tissue culture media and from other biological fluids, including human serum. To further demonstrate the utility of this approach, we investigated metabolic, transcriptomic, and metallomic responses of HEK293 cells cultured in medium depleted of Zn(II) using S100A12...
January 16, 2018: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/29334195/exoproteome-profiling-reveals-the-involvement-of-the-foldase-prsa-in-the-cell-surface-properties-and-pathogenesis-of-staphylococcus-aureus
#15
Mei-Hui Lin, Chi-Chun Li, Jwu-Ching Shu, Hao-Wei Chu, Chao-Chin Liu, Chih-Ching Wu
Staphylococcus aureus is a bacterial pathogen that produces and exports many virulence factors that cause diseases in human. PrsA, a membrane-bound foldase, is expressed ubiquitously in Gram-positive bacteria and required for the folding of exported proteins into a stable and active structure. To understand the involvement of PrsA in post-translocational protein folding in S. aureus, a PrsA-deficient mutant of S. aureus HG001 was constructed. Using isobaric tags for relative and absolute quantification (iTRAQ)-based mass spectrometry analyses, the exoproteomes of PrsA mutant and wild type S...
January 15, 2018: Proteomics
https://www.readbyqxmd.com/read/29329987/quantitative-proteomics-in-friedreich-s-ataxia-b-lymphocytes-a-valuable-approach-to-decipher-the-biochemical-events-responsible-for-pathogenesis
#16
Lorène Télot, Elodie Rousseau, Emmanuel Lesuisse, Camille Garcia, Bastien Morlet, Thibaut Léger, Jean-Michel Camadro, Valérie Serre
Friedreich's ataxia (FRDA) represents the most frequent type of autosomal-recessively inherited ataxia and is caused by the deficiency of frataxin, a mitochondrial protein. It is known that frataxin-deficiency leads to alterations in cellular and mitochondrial iron metabolism and impacts in the cell physiology at several levels. Frataxin is thought to play a role in iron-sulfur cluster biogenesis and heme synthesis. Currently, cellular antioxidant defense is dysregulated when frataxin is deficient, which exacerbates oxidative damage in FRDA...
January 9, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29329433/rage-mediates-a%C3%AE-accumulation-in-a-mouse-model-of-alzheimer-s-disease-via-modulation-of-%C3%AE-and-%C3%AE-secretase-activity
#17
Fang Fang, Qing Yu, Ottavio Arancio, Doris Chen, Smruti S Gore, Shi Fang Yan, Shirley ShiDu Yan
Receptor for Advanced Glycation End products (RAGE) has been implicated in amyloid β-peptide (Aβ)-induced perturbation relevant to the pathogenesis of Alzheimer's disease (AD). However, whether and how RAGE regulates Aβ metabolism remains largely unknown. Aβ formation arises from aberrant cleavage of amyloid precursor protein (APP) by β- and γ-secretase. To investigate whether RAGE modulates β- and γ-secretase activity potentiating Aβ formation, we generated mAPP mice with genetic deletion of RAGE (mAPP/RO)...
January 10, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29328466/knockdown-of-immature-colon-carcinoma-transcript%C3%A2-1-induces-suppression-of-proliferation-s-phase-arrest-and-apoptosis-in-leukemia-cells
#18
Guang-Yao Li, Ji-Zhu Liu, Li Zhang, Guo-Zhen Liu, Shuang-Jing Li, Tai-Wu Xiao, Jing-Xia Wang, Le-Xin Wang, Ming Hou
Immature colon carcinoma transcript 1 (ICT1), a human mitochondrial translation release factor, is a ribosome-dependent codon-independent peptidyl-tRNA hydrolase. ICT1-deficiency has been recognized as a cell growth inhibitor of hepatoblastoma and glioblastoma multiforme. To explore the role of ICT1 in human leukemia, 2 short hairpin RNAs (shRNAs) targeting ICT1 sequences were designed in leukemia U937 cells. The successful infection of ICT1 in the U937 cells was observed under a fluorescence microscope and further quantified by western blotting and quantitative real-time PCR (qRT-PCR) analysis...
January 3, 2018: Oncology Reports
https://www.readbyqxmd.com/read/29327467/erbb3-binding-protein-ebp1-is-a-novel-dppa4-cofactor-in-human-pluripotent-cells
#19
Priyanka Somanath, Kelly M Bush, Paul S Knoepfler
Developmental Pluripotency-Associated-4 (DPPA4) is one of the few core pluripotency genes lacking clearly defined molecular and cellular functions. Here we used a proteomics screening approach of human embryonic stem cell (hESC) nuclear extract to determine DPPA4 molecular functions through identification of novel cofactors. Unexpectedly, the signaling molecule ERBB3-binding protein 1 (EBP1) was the strongest candidate binding partner for DPPA4 in hESC. EBP1 is a growth factor signaling mediator present in two isoforms, p48 and p42...
January 12, 2018: Stem Cells
https://www.readbyqxmd.com/read/29324469/parafibromin-deficient-hpt-jt-type-cdc73-mutated-parathyroid-tumors-demonstrate-distinctive-morphologic-features
#20
Anthony J Gill, Grace Lim, Veronica K Y Cheung, Juliana Andrici, Joanna L Perry-Keene, Julie Paik, Loretta Sioson, Adele Clarkson, Amy Sheen, Catherine Luxford, Marianne S Elston, Goswin Y Meyer-Rochow, M Teresa Nano, Schelto Kruijff, Anton F Engelsman, Mark Sywak, Stanley B Sidhu, Leigh W Delbridge, Bruce G Robinson, Deborah J Marsh, Christopher W Toon, Angela Chou, Roderick J Clifton-Bligh
The gene CDC73 (previously known as HRPT2) encodes the protein parafibromin. Biallelic mutation of CDC73 is strongly associated with malignancy in parathyroid tumors. Heterozygous germline mutations cause hyperparathyroidism jaw tumor syndrome,which is associated with a high life-time risk of parathyroid carcinoma. Therefore loss of parafibromin expression by immunohistochemistry may triage genetic testing for hyperparathyroidism jaw tumor syndrome and be associated with malignant behavior in atypical parathyroid tumors...
January 10, 2018: American Journal of Surgical Pathology
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