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Complement deficiency

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https://www.readbyqxmd.com/read/29156781/toxoplasma-gondii-clp-family-protein-tgclpb1-plays-a-crucial-role-in-thermotolerance
#1
Shinuo Cao, Nali Du, Heming Chen, Yu Pang, Zhaoxia Zhang, Jun Zheng, Honglin Jia
Caseinolytic peptidase B (ClpB) plays a pivotal role in suppressing and reversing protein aggregation. Toxoplasma gondii is an intracellular parasitic protozoan that infects a wide variety of mammals and birds and therefore is exposed to a broad range of living condition. We screened ToxoDB (http://ToxoDB.org) and identified 10 putative T. gondii genes encoding members of the Clp superfamily of caseinolytic proteases and chaperones. Of these, we focused on characterizing the Class I ATP-dependent molecular chaperones TgClpB1, TgClpB2, and TgClpB3...
October 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29154963/macrophage-scavenger-receptor-1-contributes-to-pathogenesis-of-fulminant-hepatitis-via-neutrophil-mediated-complement-activation
#2
Yuan Tang, Huifang Li, Junru Li, Yunzhi Liu, Yanli Li, Jing Zhou, Jia Zhou, Xiao Lu, Wei Zhao, Jinlin Hou, Xiang-Yang Wang, Zhengliang Chen, Daming Zuo
BACKGROUND & AIMS: The macrophage scavenger receptor 1 (Msr1, also called SRA)is a pattern recognition receptor primarily expressed on myeloid cells, playing an important role in the maintenance of immune homeostasis. Since MSR1 expression was up-regulated in the livers of patients with fulminant hepatitis (FH), we investigated the functional mechanism of Msr1 as related to FH pathogenesis. METHODS: Msr1-deficient (Msr1(-/-)) mice and their wild-type (WT) littermates were infected with mouse hepatitis virus strain-A59 (MHV-A59) to induce FH, and the levels of tissue damage, serum alanine aminotransferase (ALT), inflammatory cytokines and complement C5a were measured and compared...
November 14, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29138283/frequency-of-and-reasons-for-paroxysmal-nocturnal-haemoglobinuria-screening-in-patients-with-unexplained-anaemia
#3
James T England, Bakul Dalal, Heather A Leitch
Referral to hematology for anemia is common. In paroxysmal nocturnal hemoglobinuria (PNH), cells deficient in the glycosylphosphatidyl inositol (GPI) anchor are lysed by complement. Eculizumab improves overall survival and quality of life while reducing hemolysis, transfusion requirements, and thrombosis. We evaluated the frequency of screening for PNH in patients with unexplained anemia. Key clinical features, laboratory data, and investigations were recorded for patients referred for anemia since 2010, without a specific cause found...
November 14, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29130721/the-nutritional-limitations-of-plant-based-beverages-in-infancy-and-childhood
#4
Isidro Vitoria
Breastfeeding, infant formula and cow's milk are basic foods in infant nutrition. However, they are being increasingly replaced either totally or partially by plant-based beverages.The composition of 164 plant-based beverages available in Spain was reviewed based on the nutritional labeling of the package and the manufacturers' webpages. This was compared to the composition of cow's milk and infant formula. In addition, the nutritional disease associated with consumption of plant-based beverages in infants and children was reviewed by means of a literature search in Medline and Embase since 1990 based on the key words "plant-based beverages" or "rice beverages" or "almond beverages" or "soy beverages" and "infant" or "child"...
October 24, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29129586/mannose-binding-lectin-and-its-roles-in-immune-responses-in-grass-carp-ctenopharyngodon-idella-against-aeromonas-hydrophila
#5
Yunfei Dang, Xinzhan Meng, Shentong Wang, Lisen Li, Meng Zhang, Moyan Hu, Xiaoyan Xu, Yubang Shen, Liqun Lv, Rongquan Wang, Jiale Li
The complement system is a crucial component of the innate immune system that links innate and adaptive immunity via four pathways. Mannose-binding lectin (MBL), the initiating molecule of the lectin pathway, plays a significant role in the innate immune system in mammals and fish. Herein, we identified an MBL homolog (gcMBL) in grass carp (Ctenopharyngodon idella). The full-length 948 bp gcMBL cDNA includes a 741 bp open reading frame encoding a 246 amino acid protein with a signal peptide, collagen triple helix repeat domain, and a C-type lectin-like/link domain...
November 10, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29125376/extending-julius-seizure-a-bang-sensitive-gene-as-a-model-for-studying-epileptogenesis-cold-shock-and-a-new-insertional-mutation
#6
Derek Dean, Hannah Weinstein, Seema Amin, Breelyn Karno, Emma McAvoy, Ronald Hoy, Andrew Recknagel, Casey Jarvis, David Deitcher
The bang-sensitive (BS) mutants of Drosophila are an important model for studying epilepsy. We recently identified a novel BS locus, julius seizure (jus), encoding a protein containing two transmembrane domains and an extracellular cysteine-rich loop. We also determined that jus(sda)(iso7.8), a previously identified BS mutation, is an allele of jus by recombination, deficiency mapping, complementation testing, and genetic rescue. RNAi knockdown revealed that jus expression is important in cholinergic neurons and that the critical stage of jus expression is the mid-pupa...
November 10, 2017: Fly
https://www.readbyqxmd.com/read/29118090/complement-activation-via-a-c3a-receptor-pathway-alters-cd4-t-lymphocytes-and-mediates-lung-cancer-progression
#7
Jeff W Kwak, Jennifer Laskowski, Howard Y Li, Maria V McSharry, Trisha R Sippel, Bonnie L Bullock, Amber M Johnson, Joanna M Poczobutt, Alexander J Neuwelt, Stephen P Malkoski, Mary C Weiser-Evans, John Lambris, Eric T Clambey, Joshua M Thurman, Raphael A Nemenoff
The complement cascade is a part of the innate immune system which acts primarily to remove pathogens and injured cells. However, complement activation is also peculiarly associated with tumor progression. Here we report mechanistic insights into this association in multiple immunocompetent orthotopic models of lung cancer. After tumor engraftment, we observed systemic activation of the complement cascade as reflected by elevated levels of the key regulator C3a. Notably, growth of primary tumors and metastases was both strongly inhibited in C3-deficient mice (C3-/- mice), with tumors undetectable in many subjects...
November 8, 2017: Cancer Research
https://www.readbyqxmd.com/read/29114257/the-rice-high-affinity-k-transporter-oshkt2-4-mediates-mg-2-homeostasis-under-high-mg-2-conditions-in-transgenic-arabidopsis
#8
Chi Zhang, Hejuan Li, Jiayuan Wang, Bin Zhang, Wei Wang, Hongxuan Lin, Sheng Luan, Jiping Gao, Wenzhi Lan
Rice (Oryza sativa; background Nipponbare) contains nine HKT (high-affinity K(+) transport)-like genes encoding membrane proteins belonging to the superfamily of Ktr/TRK/HKT. OsHKTs have been proposed to include four selectivity filter-pore-forming domains homologous to the bacterial K(+) channel KcsA, and are separated into OsHKT1s with Na(+)-selective activity and OsHKT2s with Na(+)-K(+) symport activity. As a member of the OsHKT2 subfamily, OsHKT2;4 renders Mg(2+) and Ca(2+) permeability for yeast cells and Xenopus laevis oocytes, besides K(+) and Na(+)...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/29113906/human-plasma-c3-is-essential-for-the-development-of-memory-b-but-not-t-lymphocytes
#9
Anaïs Jiménez-Reinoso, Ana V Marin, Marta Subias, Alberto López-Lera, Elena Román-Ortiz, Kathryn Payne, Cindy S Ma, Giuseppina Arbore, Martin Kolev, Simon J Freeley, Claudia Kemper, Stuart G Tangye, Edgar Fernández-Malavé, Santiago Rodríguez de Córdoba, Margarita López-Trascasa, José R Regueiro
Primary or secondary plasma C3 deficiency due to mutations in C3 or in complement Factor I impairs memory B, but not T, cell differentiation, but does not preclude intracellular C3 fragment expression in lymphocytes.
November 4, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29113537/systemic-lupus-erythematosus-with-c1q-deficiency-treatment-with-fresh-frozen-plasma
#10
Z Ekinci, K Ozturk
Treatment and outcome of systemic lupus erythematosus (SLE) in C1q deficient patients are rarely reported. The aim of this report is to share our experience about the course of management of three cases diagnosed as SLE with C1q deficiency, in light of present literature. Initial and dominant complaints of three cases from two different families were cutaneous manifestations. One patient was also diagnosed with arthritis and thrombocytopenia. Antinuclear antibody was positive in all cases, whereas anti-dsDNA was negative with normal levels of complement C3, C4 and decreased CH50 activity...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/29111970/complementation-of-a-metk-deficient-e-coli-strain-with-heterologous-adomet-synthetase-genes
#11
Gwenn G Parungao, Mojun Zhao, Qinzhe Wang, Stephen P Zano, Ronald E Viola, Robert M Blumenthal
S-adenosyl-l-methionine (AdoMet) is an essential metabolite, playing a wide variety of metabolic roles. The enzyme that produces AdoMet from l-methionine and ATP (methionine adenosyltransferase, MAT) is thus an attractive target for anti-cancer and antimicrobial agents. It would be very useful to have a system that allows rapid identification of species-specific inhibitors of this essential enzyme. A previously generated E. coli strain, lacking MAT (∆metK) but containing a heterologous AdoMet transporter, was successfully complemented with heterologous metK genes from several bacterial pathogens, as well as with MAT genes from a fungal pathogen and Homo sapiens...
November 7, 2017: Microbiology
https://www.readbyqxmd.com/read/29109286/circadian-clock-cryptochrome-proteins-regulate-autoimmunity
#12
Qi Cao, Xuan Zhao, Jingwen Bai, Sigal Gery, Haibo Sun, De-Chen Lin, Qi Chen, Zhengshan Chen, Lauren Mack, Henry Yang, Ruishu Deng, Xianping Shi, Ling-Wa Chong, Han Cho, Jianjun Xie, Quan-Zhen Li, Markus Müschen, Annette R Atkins, Christopher Liddle, Ruth T Yu, Serhan Alkan, Jonathan W Said, Ye Zheng, Michael Downes, Ronald M Evans, H Phillip Koeffler
The circadian system regulates numerous physiological processes including immune responses. Here, we show that mice deficient of the circadian clock genes Cry1 and Cry2 [Cry double knockout (DKO)] develop an autoimmune phenotype including high serum IgG concentrations, serum antinuclear antibodies, and precipitation of IgG, IgM, and complement 3 in glomeruli and massive infiltration of leukocytes into the lungs and kidneys. Flow cytometry of lymphoid organs revealed decreased pre-B cell numbers and a higher percentage of mature recirculating B cells in the bone marrow, as well as increased numbers of B2 B cells in the peritoneal cavity of Cry DKO mice...
November 6, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29107952/hereditary-angioedema-assessing-the-hypothesis-for-underlying-autonomic-dysfunction
#13
Maddalena A Wu, Francesco Casella, Francesca Perego, Chiara Suffritti, Nada Afifi Afifi, Eleonora Tobaldini, Andrea Zanichelli, Chiara Cogliati, Nicola Montano, Marco Cicardi
BACKGROUND: Attacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes. OBJECTIVE: Our study evaluates the relationship between autonomic nervous system (ANS) and contact/complement system activation. METHODS: Twenty-three HAE patients (6 males, mean age 47.5±11.4 years) during remission and 24 healthy controls (8 males, mean age 45.3±10.6 years) were studied. ECG, beat-by-beat blood pressure, respiratory activity were continuously recorded during rest (10') and 75-degrees-head-up tilt (10')...
2017: PloS One
https://www.readbyqxmd.com/read/29103803/cd39-and-cd73-activity-are-protective-in-a-mouse-model-of-antiphospholipid-antibody-induced-miscarriages
#14
Anushka N Samudra, Karen M Dwyer, Carly Selan, Susanna Freddi, Lisa Murray-Segal, Mandana Nikpour, Michael J Hickey, Karlheinz Peter, Simon C Robson, Maithili Sashindranath, Peter J Cowan, Harshal H Nandurkar
OBJECTIVE: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder of young adults associated with devastating pregnancy complications (recurrent miscarriages, preeclampsia and low birth weight) and vascular complications including thrombosis. The key components implicated in pathogenesis of APS are the complement cascade and tissue factor (TF) activity causing inflammation and coagulation. Purinergic signalling involving catabolism of ATP to adenosine by cell-surface enzymes CD39 and CD73 has anti-inflammatory and anti-thrombotic effects...
November 2, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29102589/gonococcal-arthritis-and-c2-deficiency
#15
Diego Benavent Núñez, Carolina Tornero Marín, Gema Bonilla Hernán, Adela García Perea, Alejandro Balsa Criado, Alicia Rico Nieto
Disseminated gonococcal infection is a rare presentation of the sexually transmitted pathogen, Neisseria gonorrhoeae. Here, we report the case of a 64-year-old woman with disseminated gonococcal infection, which started with symptoms of oligoarthritis and malaise. Neisseria gonorrhoeae was identified in the carpal synovial fluid. The follow-up study revealed an absence of total hemolytic complement and complement C2 was not detected. Being relatively common, C2 deficiency has been associated with disseminated gonococcal infection in a few cases...
November 1, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/29100097/monogenic-lupus-it-s-all-new
#16
REVIEW
Patricia Costa-Reis, Kathleen E Sullivan
Monogenic lupus is rare, but its study has contributed immensely to a better understanding of the pathogenesis of systemic lupus erythematosus. The first forms identified were inherited complement deficiencies, which predisposed to lupus due to impaired tolerance, and aberrant clearance of apoptotic bodies and immune complexes. In recent years, several new monogenic disorders with a lupus-like phenotype have been described. These include forms that affect nucleic acid repair, degradation and sensing (TREX1, DNASE1L3), the type I interferon (IFN) pathway (SAMHD1, RNASEH2ABC, ADAR1, IFIH1, ISG15, ACP5, TMEM173) and B cell development checkpoints (PRKCD; RAG2)...
October 27, 2017: Current Opinion in Immunology
https://www.readbyqxmd.com/read/29097367/sex-chromosome-complement-defines-diffuse-versus-focal-angiotensin-ii-induced-aortic-pathology
#17
Yasir Alsiraj, Sean E Thatcher, Eric Blalock, Bradley Fleenor, Alan Daugherty, Lisa A Cassis
OBJECTIVE: Aortic pathologies exhibit sexual dimorphism, with aneurysms in both the thoracic and abdominal aorta (ie, abdominal aortic aneurysm [AAA]) exhibiting higher male prevalence. Women have lower prevalence of aneurysms, but when they occur, aneurysms progress rapidly. To define mechanisms for these sex differences, we determined the role of sex chromosome complement and testosterone on the location and progression of angiotensin II (AngII)-induced aortic pathologies. APPROACH AND RESULTS: We used transgenic male mice expressing Sry on an autosome to create Ldlr (low-density lipoprotein receptor)-deficient male mice with an XY or XX sex chromosome complement...
November 2, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/29093712/factor-h-related-fhr-1-and-fhr-2-form-homo-and-heterodimers-while-fhr-5-circulates-only-as-homodimer-in-human-plasma
#18
Anna E van Beek, Richard B Pouw, Mieke C Brouwer, Gerard van Mierlo, Judy Geissler, Pleuni Ooijevaar-de Heer, Martin de Boer, Karin van Leeuwen, Theo Rispens, Diana Wouters, Taco W Kuijpers
The complement factor H-related (FHR) proteins are hypothesized to fine-tune the regulatory role of complement factor H (FH) in the alternative pathway of the complement system. Moreover, FHR-1, FHR-2, and FHR-5 have been proposed to be dimers, which further complicates accurate analysis. As FHRs are highly similar among themselves and toward FH, obtaining specific reagents for quantification of serum levels and functional analysis is challenging. In this study, we generated antibodies and developed ELISAs to measure FHR-1, FHR-2, and FHR-5 in serum...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29093709/age-related-macular-degeneration-a-connection-between-human-herpes-virus-6a-induced-cd46-downregulation-and-complement-activation
#19
Walter Fierz
Viruses are able to interfere with the immune system by docking to receptors on host cells that are important for proper functioning of the immune system. A well-known example is the human immunodeficiency virus that uses CD4 cell surface molecules to enter host lymphocytes and thereby deleteriously destroying the helper cell population of the immune system. A more complicated mechanism is seen in multiple sclerosis (MS) where human herpes virus-6A (HHV-6A) infects astrocytes by docking to the CD46 surface receptor...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29093663/selection-and-characterization-of-palmitic-acid-responsive-patients-with-an-oxphos-complex-i-defect
#20
Tom E J Theunissen, Mike Gerards, Debby M E I Hellebrekers, Florence H van Tienen, Rick Kamps, Suzanne C E H Sallevelt, Elvira N M M-D Hartog, Hans R Scholte, Robert M Verdijk, Kees Schoonderwoerd, Irenaeus F M de Coo, Radek Szklarczyk, Hubert J M Smeets
Mitochondrial disorders are genetically and clinically heterogeneous, mainly affecting high energy-demanding organs due to impaired oxidative phosphorylation (OXPHOS). Currently, effective treatments for OXPHOS defects, with complex I deficiency being the most prevalent, are not available. Yet, clinical practice has shown that some complex I deficient patients benefit from a high-fat or ketogenic diet, but it is unclear how these therapeutic diets influence mitochondrial function and more importantly, which complex I patients could benefit from such treatment...
2017: Frontiers in Molecular Neuroscience
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