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protein S deficiency

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https://www.readbyqxmd.com/read/29133525/optineurin-promotes-autophagosome-formation-by-recruiting-the-autophagy-related-atg12-5-16l1-complex-to-phagophores-containing-the-wipi2-protein
#1
Megha Bansal, Shivranjani C Moharir, S Purnima Sailasree, Kapil Sirohi, Cherukuri Sudhakar, D Partha Sarathi, B Jyothi Lakshmi, Mario Buono, Satish Kumar, Ghanshyam Swarup
Autophagy is a quality-control mechanism that helps to maintain cellular homeostasis by removing damaged proteins and organelles through lysosomal degradation. During autophagy, signaling events lead to the formation of a cup-shaped structure called the phagophore that matures into the autophagosome. Recruitment of the autophagy-associated Atg12-5-16L1 complex to wipi2-positive phagophores is crucial for producing LC3-II, which is required for autophagosome formation. Here, we explored the role of the autophagy receptor optineurin (Optn) in autophagosome formation...
November 13, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29127199/the-full-length-interleukin-33-flil33-importin-5-interaction-does-not-regulate-flil33-s-nuclear-localization-but-controls-its-intracellular-degradation
#2
Andrew Clerman, Zahid Noor, Rita Fishelevich, Virginia Lockatell, Brian S Hampton, Nirav G Shah, Mariah V Salcedo, Nevins W Todd, Sergei P Atamas, Irina G Luzina
Human mature interleukin-33 (MIL33) is a member of the IL-1 family and a potent regulator of immunity through its pro-T helper cell 2 (Th2) activity. Its precursor form, full-length interleukin-33 (FLIL33), is an intranuclear protein in many cell types, including fibroblasts, and its intracellular levels can change in response to stimuli. However, the mechanisms controlling the nuclear localization of FLIL33 or its stability in cells are not understood. Here, we identified importin-5 (IPO5), a member of the importin family of nuclear transport proteins, as an intracellular binding partner of FLIL33...
November 10, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29127147/igg1-is-required-for-optimal-protection-after-immunization-with-the-purified-porin-ompd-from-salmonella-typhimurium
#3
Yang Zhang, Coral Dominguez-Medina, Nicola J Cumley, Jennifer N Heath, Sarah J Essex, Saeeda Bobat, Anna Schager, Margaret Goodall, Sven Kracker, Christopher D Buckley, Robin C May, Robert A Kingsley, Calman A MacLennan, Constantino López-Macías, Adam F Cunningham, Kai-Michael Toellner
In mice, the IgG subclass induced after Ag encounter can reflect the nature of the Ag. Th2 Ags such as alum-precipitated proteins and helminths induce IgG1, whereas Th1 Ags, such as Salmonella Typhimurium, predominantly induce IgG2a. The contribution of different IgG isotypes to protection against bacteria such as S. Typhimurium is unclear, although as IgG2a is induced by natural infection, it is assumed this isotype is important. Previously, we have shown that purified S. Typhimurium porins including outer membrane protein OmpD, which induce both IgG1 and IgG2a in mice, provide protection to S...
November 10, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29126301/coagulation-testing-in-the-core-laboratory
#4
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29125223/perinatal-nutrition-interacts-with-genetic-background-to-alter-behavior-in-a-parent-of-origin-dependent-manner-in-adult-collaborative-cross-mice
#5
S A Schoenrock, D Oreper, J Farrington, R C McMullan, R Ervin, D R Miller, F Pardo-Manuel de Villena, W Valdar, L M Tarantino
Previous studies in animal models and humans have shown that exposure to nutritional deficiencies in the perinatal period increases the risk of psychiatric disease. Less well understood is how such effects are modulated by the combination of genetic background and parent-of-origin. To explore this, we exposed female mice from 20 Collaborative Cross (CC) strains to either protein deficient, vitamin D deficient, methyl donor enriched or standard diet during the perinatal period. These CC females were then crossed to a male from a different CC strain to produce reciprocal F1 hybrid females comprising 10 distinct genetic backgrounds...
November 10, 2017: Genes, Brain, and Behavior
https://www.readbyqxmd.com/read/29122468/an-intronic-variation-in-slc52a1-causes-exon-skipping-and-transient-riboflavin-responsive-multiple-acyl-coa-dehydrogenation-deficiency
#6
Signe Mosegaard, Gitte Hoffmann Bruun, Karen Freund Flyvbjerg, Yngve Thomas Bliksrud, Niels Gregersen, Maja Dembic, Ellen Annexstad, Trine Tangeraas, Rikke Katrine Jentoft Olsen, Brage S Andresen
Vitamin B2, riboflavin is essential for cellular function, as it participates in a diversity of redox reactions central to human metabolism, through its role as precursor for the cofactors flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD), which are electron carriers. The electron transfer flavoprotein (ETF) and its dehydrogenase (ETFDH), uses FAD as cofactor. The ETF and ETFDH are forming the electron transport pathway for many mitochondrial flavoprotein dehydrogenases involved in fatty acid, amino acid and choline metabolism...
November 2, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29120956/protein-kinase-c%C3%AE-deletion-causes-hypotension-and-decreased-vascular-contractility
#7
Brandi M Wynne, Cameron G McCarthy, Theodora Szasz, Patrick A Molina, Arlene B Chapman, R Clinton Webb, Janet D Klein, Robert S Hoover
AIM: Protein kinase Cα (PKCα) is a critical regulator of multiple cell signaling pathways including gene transcription, posttranslation modifications and activation/inhibition of many signaling kinases. In regards to the control of blood pressure, PKCα causes increased vascular smooth muscle contractility, while reducing cardiac contractility. In addition, PKCα has been shown to modulate nephron ion transport. However, the role of PKCα in modulating mean arterial pressure (MAP) has not been investigated...
November 8, 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/29120745/nrp2-and-cd63-are-host-factors-for-lujo-virus-cell-entry
#8
Matthijs Raaben, Lucas T Jae, Andrew S Herbert, Ana I Kuehne, Sarah H Stubbs, Yi-Ying Chou, Vincent A Blomen, Tomas Kirchhausen, John M Dye, Thijn R Brummelkamp, Sean P Whelan
Arenaviruses cause fatal hemorrhagic disease in humans. Old World arenavirus glycoproteins (GPs) mainly engage α-dystroglycan as a cell-surface receptor, while New World arenaviruses hijack transferrin receptor. However, the Lujo virus (LUJV) GP does not cluster with New or Old World arenaviruses. Using a recombinant vesicular stomatitis virus containing LUJV GP as its sole attachment and fusion protein (VSV-LUJV), we demonstrate that infection is independent of known arenavirus receptor genes. A genome-wide haploid genetic screen identified the transmembrane protein neuropilin 2 (NRP2) and tetraspanin CD63 as factors for LUJV GP-mediated infection...
November 8, 2017: Cell Host & Microbe
https://www.readbyqxmd.com/read/29120065/biallelic-mutations-in-mitochondrial-tryptophanyl-trna-synthetase-cause-levodopa-rresponsive-infantile-onset-parkinsonism
#9
E A Burke, S J Frucht, K Thompson, L A Wolfe, T Yokoyama, M Bertoni, Y Huang, M Sincan, D R Adams, R W Taylor, W A Gahl, C Toro, M C V Malicdan
Mitochondrial aminoacyl-tRNA synthetases (mtARSs) are essential, ubiquitously expressed enzymes that covalently attach amino acids to their corresponding tRNA molecules during translation of mitochondrial genes. Deleterious variants in the mtARS genes cause a diverse array of phenotypes, many of which involve the nervous system. Moreover, distinct mutations in mtARSs often cause different clinical manifestations. Recently, the gene encoding mitochondrial tryptophanyl tRNA synthetase (WARS2) was reported to cause two different neurological phenotypes, a form of autosomal recessive intellectual disability and a syndrome of severe infantile-onset leukoencephalopathy...
November 9, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29117862/successful-medical-management-of-acute-mesenteric-ischemia-due-to-superior-mesenteric-and-portal-vein-thrombosis-in-a-27-year-old-man-with-protein-s-deficiency-a-case-report
#10
N P Osti, D N Sah, R S Bhandari
BACKGROUND: Acute mesenteric ischemia poses a diagnostic challenge due to nonspecific clinical clues and lack of awareness owing to its rarity. Ischemia due to mesenteric venous thrombosis has a good prognosis compared to arterial cause and can be managed conservatively with early diagnosis. The portomesenteric venous system is an unusual site of thrombosis in patients with protein S deficiency, and its thrombosis is an uncommon cause of acute mesenteric ischemia. CASE PRESENTATION: We present a case of a 27-year-old Mongolian man who presented with acute abdominal pain increasing in severity, and refractory to repeated attempts at treatment with a misdiagnosis of acute peptic ulcer disease...
November 9, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29115020/glycosylphosphatidylinositol-gpi-anchored-protein-deficiency-serves-as-a-reliable-reporter-of-pig-a-gene-mutation-support-from-an-in-vitro-assay-based-on-l5178y-tk-cells-and-the-cd90-2-antigen
#11
Jeffrey C Bemis, Svetlana L Avlasevich, Carson Labash, Page McKinzie, Javier Revollo, Vasily N Dobrovolsky, Stephen D Dertinger
Lack of cell surface glycosylphosphatidylinositol (GPI)-anchored protein(s) has been used as a reporter of Pig-a gene mutation in several model systems. As an extension of this work, our laboratory initiated development of an in vitro mutation assay based on the flow cytometric assessment of CD90.2 expression on the cell surface of the mouse lymphoma cell line L5178Y/Tk(+/-) . Cells were exposed to mutagenic and nonmutagenic compounds for 24 hr followed by washout and incubation for an additional 7 days. Following this mutant manifestation time, cells were labeled with fluorescent antibodies against CD90...
November 8, 2017: Environmental and Molecular Mutagenesis
https://www.readbyqxmd.com/read/29113923/effects-of-spinal-non-viral-interleukin-10-gene-therapy-formulated-with-d-mannose-in-neuropathic-interleukin-10-deficient-mice-behavioral-characterization-mrna-and-protein-analysis-in-pain-relevant-tissues
#12
Arden G Vanderwall, Shahani Noor, Melody S Sun, Jacob E Sanchez, Xuexian O Yang, Lauren L Jantzie, Nikolaos Mellios, Erin D Milligan
Studies show that spinal (intrathecal; i.t.) interleukin-10 (IL-10) gene therapy reverses neuropathic pain in animal models, and co-administration with the mannose receptor (MR; CD206) ligand d-mannose (DM) greatly improves therapeutic efficacy. However, the actions of endogenous IL-10 may be required for enduring pain control observed following i.t. IL-10 gene therapy, potentially narrowing the application of this non-viral transgene delivery approach. Here, we show that i.t. application of naked plasmid DNA expressing the IL-10 transgene co-injected with DM (DM/pDNA-IL-10) for the treatment of peripheral neuropathic pain in IL-10 deficient (IL-10 KO) mice results in a profound and prolonged bilateral pain suppression...
November 4, 2017: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/29113086/iron-deficiency-anemia-not-iron-deficiency-is-associated-with-reduced-attention-in-healthy-young-women
#13
Rebecca L Cook, Nicholas J O'Dwyer, Helen M Parker, Cheyne E Donges, Hoi Lun Cheng, Katharine S Steinbeck, Eka P Cox, Janet L Franklin, Manohar L Garg, Kieron B Rooney, Helen T O'Connor
Women of reproductive age are at increased risk for iron deficiency (ID) and iron deficiency anemia (IDA), with both implicated in decreased cognitive function (CF). Obesity may complicate this association via inflammatory-mediated ferritin elevation. This cross-sectional study examined the association between hematological iron status (iron replete (IR), ID or IDA) and CF in healthy, young (18-35 years) women of normal-weight (NW: BMI 18.5-24.9 kg/m²) or obese-weight (OB: BMI >30 kg/m²). Participants completed a validated, computer-based cognition assessment evaluating impulsivity, attention, information processing, memory and executive function; CF reported as z-scores (mean ± SD)...
November 5, 2017: Nutrients
https://www.readbyqxmd.com/read/29111769/xpc-deficiency-alters-cigarette-smoke-dna-damage-cell-fate-and-accelerates-emphysema-development
#14
Catherine R Sears, Huaxin Zhou, Matthew J Justice, Amanda J Fisher, Jacob Saliba, Isaac Lamb, Jessica Wicker, Kelly S Schweitzer, Irina Petrache
Cigarette smoke (CS) exposure is a major risk factor for the development of emphysema, a common disease characterized by loss of cells comprising the lung parenchyma. The mechanisms of cell injury leading to emphysema are not completely understood but are thought to involve persistent cytotoxic or mutagenic DNA damage induced by CS. Using complementary cell culture and mouse models of CS exposure, we investigated the role of the DNA repair protein xeroderma pigmentosum group C (XPC) on CS-induced DNA damage repair and emphysema...
November 7, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/29111221/portomesentric-and-splenic-vein-thrombosis-pmsvt-after-bariatric-surgery-a-systematic-review-of-110-patients
#15
Saeed Shoar, Alan A Saber, Rebecca Rubenstein, Saeed Safari, Stacy A Brethauer, Hassan Al-Thani, Armand P Asarian, Ali Aminian
BACKGROUND: Portomesenteric and splenic vein thrombosis (PMSVT) is a rare but potentially serious complication after bariatric surgery. No study has systematically analyzed its incidence and risk factors. OBJECTIVES: To pool the data regarding PMSVT after bariatric surgery and determine its incidence and risk factors. METHODS: A meta-analysis and systematic review was conducted to retrieve studies on PMSVT after bariatric surgery. RESULTS: A total of 41 eligible studies including 110 patients with postbariatric PMSVT were enrolled; the estimated incidence rate based on 13 studies was ...
September 14, 2017: Surgery for Obesity and related Diseases: Official Journal of the American Society for Bariatric Surgery
https://www.readbyqxmd.com/read/29111212/mcpip1-controls-allergic-airway-inflammation-by-suppressing-il-5-producing-th2-cells-through-notch-gata3-pathway
#16
Hui Peng, Huan Ning, Qinghong Wang, Wenbao Lu, Yingzi Chang, Tony T Wang, Jinping Lai, Pappachan E Kolattukudy, Rong Hou, Daniel F Hoft, Mark S Dykewicz, Jianguo Liu
BACKGROUND: Asthmatic and allergic inflammation is mediated by Th2 cytokines (IL-4, IL-5 and IL-13). Though we have learned much about how Th2 cells are differentiated, the Th2 checkpoint mechanisms remain elusive. OBJECTIVES: In this study, we investigate how monocyte chemotactic protein induced protein-1 (MCPIP1, encoded by zc3h12a gene) regulates IL-5-producing Th2 cell differentiation and Th2-mediated inflammation. METHODS: The functions of zc3h12a(-/-) CD4 T cells were evaluated by checking the expression of Th2 cytokines and transcription factors in vivo and in vitro...
October 27, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29109176/candidalysin-drives-epithelial-signaling-neutrophil-recruitment-and-immunopathology-at-the-vaginal-mucosa
#17
Jonathan P Richardson, Hubertine M E Willems, David L Moyes, Saeed Shoaie, Katherine S Barker, Shir Lynn Tan, Glen E Palmer, Bernhard Hube, Julian R Naglik, Brian M Peters
Unlike other forms of candidiasis, vulvovaginal candidiasis, caused primarily by the fungal pathogen Candida albicans, is a disease of immunocompetent and otherwise healthy women. Despite its prevalence, the fungal factors responsible for initiating symptomatic infection remain poorly understood. One of the hallmarks of vaginal candidiasis is the robust recruitment of neutrophils to the site of infection, which seemingly do not clear the fungus, but rather exacerbate disease symptomatology. Candidalysin, a newly discovered peptide toxin secreted by C...
November 6, 2017: Infection and Immunity
https://www.readbyqxmd.com/read/29109083/activated-hedgehog-gli-signaling-causes-congenital-ureteropelvic-junction-obstruction
#18
Sepideh Sheybani-Deloui, Lijun Chi, Marian V Staite, Jason E Cain, Brain J Nieman, R Mark Henkelman, Brandon J Wainwright, S Steven Potter, Darius J Bagli, Armando J Lorenzo, Norman D Rosenblum
Intrinsic ureteropelvic junction obstruction is the most common cause of congenital hydronephrosis, yet the underlying pathogenesis is undefined. Hedgehog proteins control morphogenesis by promoting GLI-dependent transcriptional activation and inhibiting the formation of the GLI3 transcriptional repressor. Hedgehog regulates differentiation and proliferation of ureteric smooth muscle progenitor cells during murine kidney-ureter development. Histopathologic findings of smooth muscle cell hypertrophy and stroma-like cells, consistently observed in obstructing tissue at the time of surgical correction, suggest that Hedgehog signaling is abnormally regulated during the genesis of congenital intrinsic ureteropelvic junction obstruction...
November 6, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29107070/inflammation-driven-colon-neoplasmatogenesis-in-upa-deficient-mice-is-associated-with-an-increased-expression-of-runx-transcriptional-regulators
#19
Hara Afaloniati, George S Karagiannis, Alexandros Hardas, Theofilos Poutahidis, Katerina Angelopoulou
Deregulation of the bone morphogenetic protein (BMP) pathway has been documented in colorectal cancer (CRC). Previously, we investigated possible associations between urokinase-type plasminogen activator (uPA) deficiency and expression of extracellular constituents of BMP signaling in a newly developed mouse model of inflammation-driven intestinal neoplasmatogenesis, in which chronic colitis and CRC are induced using dextran sodium sulfate (DSS). In this report, we explored the contribution of intracellular components of Smad-mediated BMP signal transduction using the same model...
October 26, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/29106755/canine-gm2-gangliosidosis-sandhoff-disease-associated-with-a-3-base-pair-deletion-in-the-hexb-gene
#20
P Wang, P S Henthorn, E Galban, G Lin, T Takedai, M Casal
BACKGROUND: GM2-gangliosidosis is a fatal neurodegenerative lysosomal storage disease (LSD) caused by deficiency of either β-hexosaminidase A (Hex-A) and β-hexosaminidase B (Hex-B) together, or the GM2 activator protein. Clinical signs can be variable and are not pathognomonic for the specific, causal deficiency. OBJECTIVES: To characterize the phenotype and genotype of GM2-gangliosidosis disease in an affected dog. ANIMALS: One affected Shiba Inu and a clinically healthy dog...
November 6, 2017: Journal of Veterinary Internal Medicine
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