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Complement alternative pathway

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https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#1
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29337453/-amd-and-complement-pathway-in-2017
#2
Francine Behar-Cohen
AMD is the major cause of visual loss in individuals older than 50 in the western countries. Whilst tremendous progress has been made by the intraocular repeated injections of anti-VEGFs in wet AMD, no treatment is recognized to prevent progression of dry AMD, which represents more than 50 % of the cases. Interestingly, an over activation of the complement alternative pathway has been associated to both forms of AMD. But, several clinical studies have failed to demonstrate any efficacy of molecules blocking the complement pathway at various stage of its activation...
January 10, 2018: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29335241/the-mfhr1-fusion-protein-is-a-novel-synthetic-multitarget-complement-inhibitor-with-therapeutic-potential
#3
Stefan Michelfelder, Friedericke Fischer, Astrid Wäldin, Kim V Hörle, Martin Pohl, Juliana Parsons, Ralf Reski, Eva L Decker, Peter F Zipfel, Christine Skerka, Karsten Häffner
The complement system is essential for host defense, but uncontrolled complement system activation leads to severe, mostly renal pathologies, such as atypical hemolytic uremic syndrome or C3 glomerulopathy. Here, we investigated a novel combinational approach to modulate complement activation by targeting C3 and the terminal pathway simultaneously. The synthetic fusion protein MFHR1 links the regulatory domains of complement factor H (FH) with the C5 convertase/C5b-9 inhibitory fragment of the FH-related protein 1...
January 15, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29329521/treating-c3-glomerulopathy-with-eculizumab
#4
Thomas Welte, Frederic Arnold, Julia Kappes, Maximilian Seidl, Karsten Häffner, Carsten Bergmann, Gerd Walz, Elke Neumann-Haefelin
BACKGROUND: C3 glomerulopathy (C3G) is a rare, but severe glomerular disease with grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well as inherited dysregulation of the alternative pathway of the complement cascade. Currently, there is no established therapy. Treatment with the C5 complement inhibitor eculizumab may be a therapeutic option. However, due to rarity of the disease, parameters predicting treatment response remain largely unknown. METHODS: Seven patients with C3G (five with C3 glomerulonephritis and two with dense deposit disease) were treated with eculizumab...
January 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#5
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29325709/acute-hyperoxia-induces-systemic-responses-with-no-major-changes-in-peripheral-tissues-in-the-senegalese-sole-solea-senegalensis-kaup-1858
#6
Marina Machado, Diogo Malheiro, Ana Couto, Jonathan M Wilson, Matthew Guerreiro, Rita Azeredo, Jon C Svendsen, António Afonso, Renata Serradeiro, Benjamín Costas
Senegalese sole Solea senegalensis is currently farmed in recirculation aquaculture systems that often involve water re-oxygenation, which in turn may cause acute or prolonged hyperoxia exposures. In order to understand the impact of acute hyperoxia on the fish immune system and peripheral tissues such as gills and gut, Senegalese sole juveniles (30.05 ± 1.72 g) were exposed to normoxia (100% O2sat) as control and two hyperoxic conditions (150 and 200% O2sat) and sampled at 4 and 24 h. Fish haematological profile, total and differential blood cell counts and plasma immune parameters were analysed...
January 8, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29319762/complement-system-and-c4d-expression-in-cases-of-membranous-nephropathy
#7
Fabiano Bichuette Custódio, Crislaine Aparecida da Silva, Fernanda Rodrigues Helmo, Juliana Reis Machado, Marlene Antônia Dos Reis
INTRODUCTION: Membranous nephropathy (MN) is one of the major causes of nephrotic syndrome. The complement system plays a key role in the pathophysiology of MN. OBJECTIVES: To identify the complement pathway possibly activated in MN cases and correlate the presence of C4d with more severe clinical and histological markers. METHODS: Sixty nine cases from renal biopsy with membranous nephropathy were investigated. The presence of C1q was analyzed by direct immunofluorescence; and expression of C4d by immunohistochemistry...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29314145/toward-complement-inhibition-2-0-next-generation-anti-complement-agents-for-paroxysmal-nocturnal-hemoglobinuria
#8
Antonio M Risitano, Serena Marotta
Therapeutic complement inhibition by eculizumab has revolutionized the treatment of paroxysmal nocturnal hemoglobinuria (PNH) with a major impact on its natural history. Nevertheless, emerging unmet clinical needs may benefit from the development of novel complement inhibitors. Novel strategies of complement inhibition exploit different agents targeting C5, as well as compound intercepting the complement cascade at the level of its key component C3, or even upstream at the level of components involved in complement alternative pathway initiation...
January 4, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29312858/de-novo-glomerular-diseases-after-renal-transplantation-how-is-it-different-from-recurrent-glomerular-diseases
#9
REVIEW
Fedaey Abbas, Mohsen El Kossi, Jon Kim Jin, Ajay Sharma, Ahmed Halawa
The glomerular diseases after renal transplantation can occur de novo, i.e., with no relation to the native kidney disease, or more frequently occur as a recurrence of the original disease in the native kidney. There may not be any difference in clinical features and histological pattern between de novo glomerular disease and recurrence of original glomerular disease. However, structural alterations in transplanted kidney add to dilemma in diagnosis. These changes in architecture of histopathology can happen due to: (1) exposure to the immunosuppression specifically the calcineurin inhibitors (CNI); (2) in vascular and tubulointerstitial alterations as a result of antibody mediated or cell-mediated immunological onslaught; (3) post-transplant viral infections; (4) ischemia-reperfusion injury; and (5) hyperfiltration injury...
December 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/29305301/complement-factor-h-family-proteins-in-their-non-canonical-role-as-modulators-of-cellular-functions
#10
REVIEW
Mihály Józsi, Andrea E Schneider, Éva Kárpáti, Noémi Sándor
Complement factor H is a major regulator of the alternative pathway of the complement system. The factor H-related proteins are less characterized, but recent data indicate that they rather promote complement activation. These proteins have some common ligands with factor H and have both overlapping and distinct functions depending on domain composition and the degree of conservation of amino acid sequence. Factor H and some of the factor H-related proteins also appear in a non-canonical function that is beyond their role in the modulation of complement activation...
January 2, 2018: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/29296203/the-pathogenesis-of-diclofenac-induced-immunoallergic-hepatitis-in-a-canine-model-of-liver-injury
#11
Saravanakumar Selvaraj, Jung-Hwa Oh, Reinhard Spanel, Florian Länger, Hyoung-Yun Han, Eun-Hee Lee, Seokjoo Yoon, Jürgen Borlak
Hypersensitivity to non-steroidal anti-inflammatory drugs is a common adverse drug reaction and may result in serious inflammatory reactions of the liver. To investigate mechanism of immunoallergic hepatitis beagle dogs were given 1 or 3 mg/kg/day (HD) oral diclofenac for 28 days. HD diclofenac treatment caused liver function test abnormalities, reduced haematocrit and haemoglobin but induced reticulocyte, WBC, platelet, neutrophil and eosinophil counts. Histopathology evidenced hepatic steatosis and glycogen depletion, apoptosis, acute lobular hepatitis, granulomas and mastocytosis...
December 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/29288229/genetic-variation-in-cfh-predicts-phenytoin-induced-maculopapular-exanthema-in-european-descent-patients
#12
Mark McCormack, Hongsheng Gui, Andrés Ingason, Doug Speed, Galen E B Wright, Eunice J Zhang, Rodrigo Secolin, Clarissa Yasuda, Maxwell Kwok, Stefan Wolking, Felicitas Becker, Sarah Rau, Andreja Avbersek, Kristin Heggeli, Costin Leu, Chantal Depondt, Graeme J Sills, Anthony G Marson, Pauls Auce, Martin J Brodie, Ben Francis, Michael R Johnson, Bobby P C Koeleman, Pasquale Striano, Antonietta Coppola, Federico Zara, Wolfram S Kunz, Josemir W Sander, Holger Lerche, Karl Martin Klein, Sarah Weckhuysen, Martin Krenn, Lárus J Gudmundsson, Kári Stefánsson, Roland Krause, Neil Shear, Colin J D Ross, Norman Delanty, Munir Pirmohamed, Bruce C Carleton, Fernando Cendes, Iscia Lopes-Cendes, Wei-Ping Liao, Terence J O'Brien, Sanjay M Sisodiya, Stacey Cherny, Patrick Kwan, Larry Baum, Gianpiero L Cavalleri
OBJECTIVE: To characterize, among European and Han Chinese populations, the genetic predictors of maculopapular exanthema (MPE), a cutaneous adverse drug reaction common to antiepileptic drugs. METHODS: We conducted a case-control genome-wide association study of autosomal genotypes, including Class I and II human leukocyte antigen (HLA) alleles, in 323 cases and 1,321 drug-tolerant controls from epilepsy cohorts of northern European and Han Chinese descent. Results from each cohort were meta-analyzed...
December 29, 2017: Neurology
https://www.readbyqxmd.com/read/29284023/growth-phenotype-analysis-of-heme-synthetic-enzymes-in-a-halophilic-archaeon-haloferax-volcanii
#13
Naoki Kosugi, Takuma Araki, Junpei Fujita, Satoru Tanaka, Taketomo Fujiwara
Halophilic euryarchaea lack many of the genes necessary for the protoporphyrin-dependent heme biosynthesis pathway previously identified in animals and plants. Bioinformatic analysis suggested the presence of two heme biosynthetic processes, an Fe-coproporphyrinogen III (coproheme) decarboxylase (ChdC) pathway and an alternative heme biosynthesis (Ahb) pathway, in Haloferax volcanii. PitA is specific to the halophilic archaea and has a unique molecular structure in which the ChdC domain is joined to the antibiotics biosynthesis monooxygenase (ABM)-like domain by a histidine-rich linker sequence...
2017: PloS One
https://www.readbyqxmd.com/read/29282226/maternal-and-fetal-outcomes-of-pregnancies-in-women-with-atypical-hemolytic-uremic-syndrome
#14
Martina Gaggl, Christof Aigner, Dorottya Csuka, Ágnes Szilágyi, Zoltán Prohászka, Renate Kain, Natalja Haninger, Maarten Knechtelsdorfer, Raute Sunder-Plassmann, Gere Sunder-Plassmann, Alice Schmidt
Atypical HUS (aHUS) is a disorder most commonly caused by inherited defects of the alternative pathway of complement, or the proteins that regulate this pathway, and life-threatening episodes of aHUS can be provoked by pregnancy. We retrospectively and prospectively investigated 27 maternal and fetal pregnancy outcomes in 14 women with aHUS from the Vienna Thrombotic Microangiopathy Cohort. Seven pregnancies (26%) were complicated by pregnancy-associated aHUS (p-aHUS), of which three appeared to be provoked by infection, bleeding, and curettage, and three individuals were considered to have preeclampsia/HELLP syndrome before the definitive diagnosis of p-aHUS was made...
December 27, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29279276/acquired-partial-lipodystrophy-and-c3-glomerulopathy-dysregulation-of-the-complement-system-as-a-common-pathogenic-mechanism
#15
Fernando Corvillo, Margarita López-Trascasa
The activation of the alternative pathway of the complement is involved in the development of several renal diseases, such as atypical haemolytic uremic syndrome and C3 glomerulopathy. In C3 glomerulopathy, a high percentage of patients have circulating levels of the autoantibody called C3NeF, which causes systemic dysregulation of the complement system. In some cases, the presence of this antibody has been related with abnormalities of adipose tissue, causing acquired partial lipodystrophy (Barraquer-Simons syndrome)...
December 23, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29278680/genetic-and-molecular-basis-of-the-immune-system-in-the-brachiopod-lingula-anatina
#16
Marco Gerdol, Yi-Jyun Luo, Noriyuki Satoh, Alberto Pallavicini
The extension of comparative immunology to non-model systems, such as mollusks and annelids, has revealed an unexpected diversity in the complement of immune receptors and effectors among evolutionary lineages. However, several lophotrochozoan phyla remain unexplored mainly due to the lack of genomic resources. The increasing accessibility of high-throughput sequencing technologies offers unique opportunities for extending genome-wide studies to non-model systems. As a result, the genome-based study of the immune system in brachiopods allows a better understanding of the alternative survival strategies developed by these immunologically neglected phyla...
December 23, 2017: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/29250893/atypical-hemolytic-uremic-syndrome-a-meta-analysis-of-case-reports-confirms-the-prevalence-of-genetic-mutations-and-the-shift-of-treatment-regimens
#17
Vinod Krishnappa, Mohit Gupta, Mohamed Elrifai, Bahar Moftakhar, Michael J Ensley, Tushar J Vachharajani, Sidharth Kumar Sethi, Rupesh Raina
Atypical hemolytic uremic syndrome (aHUS) is a rare life-threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta-analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations...
December 17, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29245944/extracellular-vesicles-from-kshv-infected-endothelial-cells-activate-the-complement-system
#18
Hyungtaek Jeon, Seung-Min Yoo, Hyo Sun Choi, Ji Young Mun, Hee-Gyoo Kang, Jiyeong Lee, Jinsung Park, Shou-Jiang Gao, Myung-Shin Lee
Extracellular vesicles (EVs), released by cells, are associated with cell-to-cell communication and regulate various cellular processes. EVs draw parallels with viruses for their similar structures and functions. Increasing evidences from recent studies indicate that cells infected with viruses release a variety of EVs. Delineating the functions and mechanisms of EVs released during virus infection is essential for understanding the molecular basis of viral infection and replication as well as associated pathogenesis...
November 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/29243465/atypical-hemolytic-uremic-syndrome
#19
REVIEW
Kati Kaartinen, Leena Martola, Seppo Meri
Atypical hemolytic-uremic syndrome (aHUS) is a rare form of thrombotic microagiopathy caused dysregulation of the alternative pathway of the complement resulting in tissue. In aHUS, activation of the alternative pathway of the complement is in an aberrant way directed against endothelial cells and blood cells. This is either due to a mutation in a complement factor, most commonly factor H, or an autoantibody against a complement regulator. In some patients the underlying disorder is not identified despite thorough examinations...
2017: Duodecim; Lääketieteellinen Aikakauskirja
https://www.readbyqxmd.com/read/29242567/a-coxiella-mutualist-symbiont-is-essential-to-the-development-of-rhipicephalus-microplus
#20
Melina Garcia Guizzo, Luís Fernando Parizi, Rodrigo Dutra Nunes, Renata Schama, Rodolpho M Albano, Lucas Tirloni, Daiane Patrícia Oldiges, Ricardo Pilz Vieira, Wanderson Henrique Cruz Oliveira, Milane de Souza Leite, Sergio A Gonzales, Marisa Farber, Orlando Martins, Itabajara da Silva Vaz, Pedro L Oliveira
The cattle tick Rhipicephalus microplus is a hematophagous ectoparasite that causes important economic losses in livestock. Different species of ticks harbor a symbiont bacterium of the genus Coxiella. It was showed that a Coxiella endosymbiont from R. microplus (CERM) is a vertically transmitted mutualist symbiont, comprising 98% of the 16S rRNA sequences in both eggs and larvae. Sequencing of the bacterial genome revealed genes for biosynthetic pathways for several vitamins and key metabolic cofactors that may provide a nutritional complement to the tick host...
December 14, 2017: Scientific Reports
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