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C3 complement

Iwona Żychowska, Dorota Suszek, Magdalena Dryglewska, Dorota Nurzyńska, Helena Donica, Maria Majdan
OBJECTIVE: Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease leading to chronic inflammation of numerous tissues and organs. The search for clinically useful markers of its activity is ongoing. At present, it is suggested that serum free light chains (FLC) may be useful in assessing SLE activity. The aim of study: To investigate the relationship between serum levels of free light chains (FLC) and the activity of SLE. PATIENTS AND METHODS: Material and methods: Eighty-four SLE patients (75 female; 9 men) aged 34...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Ulkem Kocoglu Barlas, Hasan Serdar Kıhtır, Nilufer Goknar, Melike Ersoy, Nihal Akcay, Esra Sevketoglu
BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury...
March 20, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Tomohiko Yamamura, Kandai Nozu, Hiroaki Ueda, Rika Fujimaru, Ryutaro Hisatomi, Yoko Yoshida, Hideki Kato, Masaomi Nangaku, Toshiyuki Miyata, Toshihiro Sawai, Shogo Minamikawa, Hiroshi Kaito, Masafumi Matsuo, Kazumoto Iijima
Pathogenic variants in specific complement-related genes lead to atypical hemolytic uremic syndrome (aHUS). Some reports have indicated that patients with digenic variants in these genes might present severer phenotypes. Upon detecting novel intronic variants, transcriptional analysis is necessary to prove pathogenicity; however, when intronic variants are located in intron 1 and, as a result, no transcript is produced, no appropriate method had been established to reveal the pathogenicity. Recently, the minigene assay was used to assess the pathogenicity of intronic variants...
March 19, 2018: Journal of Human Genetics
Sarah E Sartain, Nancy A Turner, Joel L Moake
Atypical hemolytic uremic syndrome (aHUS) and bone marrow transplantation-associated thrombotic microangiopathy (TA-TMA) are associated with excessive activation of the alternative complement pathway (AP) and with severe renal - and rarely, cerebral - microvascular damage. Here, we compared AP activation and regulation in human glomerular and brain microvascular endothelial cells (GMVECs and BMVECs, respectively) unstimulated or stimulated by the proinflammatory cytokine, tumor necrosis factor (TNF). Compared with GMVECs and under both experimental conditions, BMVECs had increased gene expression of the AP-related genes C3 , CFB , and C5 , and decreased expression of CFD This was associated with increased expression in BMVECs (relative to GMVECs) of the genes for surface and soluble regulatory molecules ( CD46 , THBD , CD55 , CFI , and CFH ) suppressing formation of the AP C3 and C5 convertases...
March 19, 2018: Journal of Biological Chemistry
Ramya Vangipuram, Tiffany Hinojosa, Daniel J Lewis, Christopher Downing, Caleb Hixson, Julio César Salas-Alanis, Stephen K Tyring
A 75-year-old African-American man presented with a 3-year history of painless, fluid-filled blisters, for which his primary care physician had treated him with doxycycline, cephalexin, and topical corticosteroids, with no significant improvement. The blisters had ruptured spontaneously and healed with scarring. He denied antecedent trauma. His medical history was remarkable for insulin-dependent type 2 diabetes mellitus, hypertension, hypercholesterolemia, primary cutaneous melanoma status-post excision, and breast cancer status-post mastectomy and chemotherapy...
2018: Skinmed
Sanjay Tewari, George Renney, John Brewin, Kate Gardner, Fenella Kirkham, Baba Inusa, James E Barrett, Stephan Menzel, Swee Lay Thein, Malcolm Ward, David C Rees
Silent cerebral infarction is the commonest neurological abnormality in children with sickle cell anemia, affecting 30-40% 14 year olds. There are no known biomarkers to identify children with silent cerebral infarcts and the pathological basis is also unknown. We used an unbiased proteomic discovery approach to identify plasma proteins differing in concentration between children with and without silent cerebral infarcts. Clinical parameters and plasma samples were analysed from 51 children (mean age 11.8 years, range 6-18) with sickle cell anemia (HbSS)...
March 15, 2018: Haematologica
Shuang-An Li, Wei-Dan Jiang, Lin Feng, Yang Liu, Pei Wu, Jun Jiang, Sheng-Yao Kuang, Ling Tang, Wu-Neng Tang, Yong-An Zhang, Juan Yang, Xu Tang, He-Qun Shi, Xiao-Qiu Zhou
In this study, we investigated the effects of dietary myo-inositol on the intestinal immune barrier function and related signaling pathway in young grass carp (Ctenopharyngodon idella). A total of 540 young grass carp (221.33 ± 0.84 g) were fed six diets containing graded levels of myo-inositol (27.0, 137.9, 286.8, 438.6, 587.7 and 737.3 mg/kg) for 10 weeks. After the growth trial, fish were challenged with Aeromonas hydrophila. The results indicated that compared with the optimal dietary myo-inositol level, myo-inositol deficiency (27...
March 12, 2018: Fish & Shellfish Immunology
Mariola A Dietrich, Piotr Hliwa, Mikołaj Adamek, Dieter Steinhagen, Halina Karol, Andrzej Ciereszko
The environmental temperature affects plasma biochemical indicators, antioxidant status and hematological and immunological parameters in fish. So far, only single blood proteins have been identified in response to temperature changes. The aim of this study was to compare the proteome of carp blood plasma from males acclimated to warm (30 °C) and cold (10 °C) temperatures by two-dimensional differential gel electrophoresis followed by MALDI-TOF/TOF mass spectrometry. A total of 47 spots were found to be differentially regulated by temperature (>1...
March 12, 2018: Fish & Shellfish Immunology
Emilio Russo, Salvatore D'Angelo, Giorgio Ammerata, Ludovico Abenavoli, Daniele Mauro, Caterina Bruno, Saverio Naty, Ignazio Olivieri, Giovambattista De Sarro, Francesco Ursini
BACKGROUND: Recent evidence demonstrated a potential role of complement C3 as a candidate biomarker of cardiometabolic risk in the general population. OBJECTIVE: Aim of the present study was to investigate the correlation between complement C3 levels and comorbid type 2 diabetes (T2DM) in rheumatoid arthritis (RA) patients. METHODS: For the present study, 40 consecutive diabetic RA patients (RA/T2DM+ group) and 80 consecutive RA patients without diabetes (RA/T2DM- group) were recruited...
March 13, 2018: Reviews on Recent Clinical Trials
Jun Kim, Hong Lan Jin, Dae Sik Jang, Kwang Won Jeong, Se-Young Choung
Age-related macular degeneration (AMD) is among the increasing number of diseases causing irreversible blindness in the elderly. Dry AMD is characterized by the accumulation of lipofuscin in retinal pigment epithelium (RPE) cells. N-Retinylidene-N-retinylethanolamine (A2E), a component of lipofuscin, is oxidized to oxo-A2E under blue light illumination, leading to retinal cell death. The aim of this study was to investigate the protective effect and mechanism of quercetin-3-O-α-l-arabinopyranoside (QA) against blue light (BL)-induced damage in both RPE cells and mice models...
March 15, 2018: Food & Function
Gustavo A Garcia, Bérenger Gans, Julia Krüger, Fabian Holzmeier, Anja Röder, Allan Lopes, Christa Fittschen, Christian Alcaraz, Jean-Christophe Loison
We present the photoelectron spectra of C3 Hx (x = 0-3) formed in a microwave discharge flow-tube reactor by consecutive H abstractions from C3 H4 (C3 Hx + F → C3 Hx-1 + HF (x = 1-4)), but also from F + CH4 schemes by secondary reactions. The spectra were obtained combining tunable VUV synchrotron radiation with double imaging electron/ion coincidence techniques, yielding mass-selected threshold photoelectron spectra. The obtained results complement not only existing ones, but for the first time the photoelectron spectra of C3 , cyclic and linear C3 H (c,l-C3 H) as well as of the excited states of C3 H3 are reported...
March 14, 2018: Physical Chemistry Chemical Physics: PCCP
Min Liu, Hui Wang, Jiaxiang Zhang, Xiaodong Yang, Bodong Li, Changhao Wu, Qixing Zhu
Both NF-κB pathway and complement activation appear to be involved in kidney damage induced by trichloroethylene (TCE). However, any relationship between these two systems has not yet been established. The present study aimed to clarify the role of NF-κB in complement activation and renal injury in TCE-sensitized BALB/c mice. Mice were sensitized by an initial subcutaneous injection and repeated focal applications of TCE to dorsal skin at specified timepoints. NF-κB inhibitor pyrrolidine dithiocarbamate (PDTC) was injected (intraperitoneal) before the final two focal TCE challenges...
December 2018: Journal of Immunotoxicology
Nilisha Fernando, Riccardo Natoli, Tanja Racic, Yvette Wooff, Jan Provis, Krisztina Valter
The complement system is highly implicated in both the prevalence and progression of Age-Related Macular Degeneration (AMD). Complement system inhibitors therefore have potential therapeutic value in managing excessive activation of the complement pathways in retinal degenerations. The vaccinia virus complement control protein (VCP) has been shown to be effective as a complement inhibitor in neuroinflammatory models including traumatic brain injury and spinal cord injury. We aimed to investigate the potential of VCP as a therapeutic molecule for retinal degenerations...
2018: PloS One
Iwona Żychowska, Dorota Suszek, Magdalena Dryglewska, Maria Majdan
BACKGROUND: Systemic lupus erythematosus (SLE) is characterized by alternating periods of activity and remission. A portion of the patients suffers from the chronically active form of the disease. The search for clinically useful markers of its activity is ongoing. At present, it is suggested that β2-microglobulin (β2M) may be useful in assessing SLE activity. OBJECTIVES: The objective of the paper was to investigate the relationship between serum β2M concentration and SLE activity...
March 13, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Sabine Buhner, Hannes Hahne, Kerstin Hartwig, Qin Li, Sheila Vignali, Daniela Ostertag, Chen Meng, Gabriele Hörmannsperger, Breg Braak, Christian Pehl, Thomas Frieling, Giovanni Barbara, Roberto De Giorgio, Ihsan Ekin Demir, Güralp Onur Ceyhan, Florian Zeller, Guy Boeckxstaens, Dirk Haller, Bernhard Kuster, Michael Schemann
BACKGROUND & AIMS: The causes of gastrointestinal complaints in irritable bowel syndrome (IBS) remain poorly understood. Altered nerve function has emerged as an important pathogenic factor as IBS mucosal biopsy supernatants consistently activate enteric and sensory neurons. We investigated the neurally active molecular components of such supernatants from patients with IBS and quiescent ulcerative colitis (UC). METHOD: Effects of supernatants from 7 healthy controls (HC), 20 IBS and 12 UC patients on human and guinea pig submucous neurons were studied with neuroimaging techniques...
2018: PloS One
Riccardo Natoli, Nilisha Fernando, Tess Dahlenburg, Haihan Jiao, Riemke Aggio-Bruce, Nigel L Barnett, Juan Manuel Chao de la Barca, Guillaume Tcherkez, Pascal Reynier, Johnny Fang, Joshua A Chu-Tan, Krisztina Valter, Jan Provis, Matt Rutar
Purpose: Systemic increases in reactive oxygen species, and their association with inflammation, have been proposed as an underlying mechanism linking obesity and age-related macular degeneration (AMD). Studies have found increased levels of oxidative stress biomarkers and inflammatory cytokines in obese individuals; however, the correlation between obesity and retinal inflammation has yet to be assessed. We used the leptin-deficient (ob/ob) mouse to further our understanding of the contribution of obesity to retinal oxidative stress and inflammation...
2018: Molecular Vision
Jean Paul Ten Klooster, Alexandros Sotiriou, Sjef Boeren, Stefan Vaessen, Jacques Vervoort, Raymond Pieters
Currently, there is a worldwide increase of patients with Type 2 diabetes (T2D). During the progression of healthy obese to T2D status, there is an influx of immune cells, in particular macrophages, into visceral adipose tissue, accompanied by an increase of inflammatory cytokines, such as, IL6, TNFα and Hp. To get a better insight in the underlying mechanisms, we performed a quantitative LCMS analysis on a modified in vitro assay, combining 3T3L1 adipocytes and activated RAW264.7 macrophages, thus mimicking inflamed adipose tissue...
March 8, 2018: Archives of Biochemistry and Biophysics
Mathieu Ing, Brian E Hew, David C Fritzinger, Sandrine Delignat, Sébastien Lacroix-Desmazes, Carl-Wilhelm Vogel, Julie Rayes
Cobra venom factor (CVF) is the complement-activating protein in cobra venom. Humanized CVF (hCVF) is a human C3 derivative where the C-terminal 168 amino acid residues were replaced with the homologous sequence from CVF. hCVF has been shown in multiple models of disease with complement pathology to be a promising therapeutic agent, with no observed adverse effects. Here we describe the antibody response to hCVF in two different strains of mice. hCVF was able to repeatedly decomplement the mice after four injections in weekly intervals, demonstrating the absence of a neutralizing antibody response...
March 8, 2018: Molecular Immunology
Nitesh Mishra, Madhav Mohata, Heena Aggarwal, Omkar Chaudhary, Bimal Kumar Das, Sanjeev Sinha, Anjali Hazarika, Kalpana Luthra
During infection and budding, human immunodeficiency virus-1 (HIV-1) acquires regulators of Complement Activation (RCAs) along with the host cell membrane on the viral envelope. Activation of host complement system results in opsonization of virus by complement fragments, however the virus evades complement mediated lysis (CoML) by virtue of the RCAs on the viral envelope. The RCAs on HIV-1 envelope process complement protein C3 into various fragments that promote viral entry and infection of cells through different complement receptors...
March 7, 2018: Molecular Immunology
Miriam Steinberg, Joseph P Gaut, Stanley Paul Hmiel, Aadil Kakajiwala
Monoclonal gammopathies are a rare diagnosis in pediatric patients. A 19-year-old female patient with past medical history of hypogammaglobulinemia and natural killer cell deficiency and stage III follicular lymphoma, in remission, presented with a right-sided pneumonia, noted to have acute kidney injury and proteinuria. Complement C3 and C4 levels were normal. Anti-double-stranded DNA antibodies, antinuclear antibodies, anti-extractable nuclear antigen antibodies, and antineutrophil cytoplasmic antibodies were negative...
March 9, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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