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Complement deficiency

Xiaobo Wu, Irina Hutson, Antonina M Akk, Smita Mascharak, Christine T N Pham, Dennis E Hourcade, Rebecca Brown, John P Atkinson, Charles A Harris
Factor D (FD) is an essential component of the complement alternative pathway (AP). It is an attractive pharmaceutical target because it is an AP-specific protease circulating in blood. Most components of the complement activation pathways are produced by the liver, but FD is highly expressed by adipose tissue. Two critical questions are: 1) to what degree does adipose tissue contribute to circulating FD levels and 2) what quantity of FD is sufficient to maintain a functional AP? To address these issues, we studied a novel mouse strain with complete lipodystrophy (LD), the fld mouse with partial LD, an FD-deficient mouse, and samples from lipodystrophic patients...
March 12, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Roan Louw, Izelle Smuts, Kimmey-Li Wilsenach, Lindi-Maryn Jonck, Maryke Schoonen, Francois H van der Westhuizen
BACKGROUND: Coenzyme Q10 (CoQ10 ) is an important component of the mitochondrial respiratory chain (RC) and is critical for energy production. Although the prevalence of CoQ10 deficiency is still unknown, the general consensus is that the condition is under-diagnosed. The aim of this study was to retrospectively investigate CoQ10 deficiency in frozen muscle specimens in a cohort of ethnically diverse patients who received muscle biopsies for the investigation of a possible RC deficiency (RCD)...
February 23, 2018: Molecular Genetics and Metabolism
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
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
Manoj Kumar, Laxmi Mishra, Paul Carr, Michael Pilling, Peter Gardner, Shawn D Mansfield, Simon R Turner
Cellulose microfibrils are the basic units of cellulose in plants. The structure of these microfibrils is at least partly determined by the structure of the cellulose synthase complex. In higher plants, this complex is composed of 18 to 24 catalytic subunits known as CELLULOSE SYNTHASE A (CESA) proteins. Three different classes of CESA proteins are required for cellulose synthesis and for secondary cell wall cellulose biosynthesis, which include CESA4, CESA7, and CESA8. To probe the relationship between CESA proteins and microfibril structure, we created mutant cesa proteins that lack catalytic activity but retain sufficient structural integrity to allow assembly of the cellulose synthase complex...
March 9, 2018: Plant Physiology
Marieke F Fransen, Hreinn Benonisson, Wendy W van Maren, Heng Sheng Sow, Cor Breukel, Margot M Linssen, Jill W C Claassens, Conny Brouwers, Jos van der Kaa, Marcel Camps, Jan Willem Kleinovink, Kelly K Vonk, Sandra van Heiningen, Ngaisah Klar, Lianne van Beek, Vanessa van Harmelen, Lucia Daxinger, Kutty S Nandakumar, Rikard Holmdahl, Chris Coward, Qingshun Lin, Sachiko Hirose, Daniela Salvatori, Thorbald van Hall, Cees van Kooten, Piero Mastroeni, Ferry Ossendorp, J Sjef Verbeek
By their interaction with IgG immune complexes, FcγR and complement link innate and adaptive immunity, showing functional redundancy. In complement-deficient mice, IgG downstream effector functions are often impaired, as well as adaptive immunity. Based on a variety of model systems using FcγR-knockout mice, it has been concluded that FcγRs are also key regulators of innate and adaptive immunity; however, several of the model systems underpinning these conclusions suffer from flawed experimental design. To address this issue, we generated a novel mouse model deficient for all FcγRs (FcγRI/II/III/IV-/- mice)...
March 9, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Christopher C T Sng, Sorcha O'Byrne, Daniil M Prigozhin, Matthias R Bauer, Jennifer C Harvey, Michelle Ruhle, Ben G Challis, Sara Lear, Lee D Roberts, Sarita Workman, Tobias Janowitz, Lukasz Magiera, Rainer Doffinger, Matthew S Buckland, Duncan J Jodrell, Robert K Semple, Timothy J Wilson, Yorgo Modis, James E D Thaventhiran
No abstract text is available yet for this article.
March 6, 2018: Journal of Allergy and Clinical Immunology
Katharina Bröker, Julia Figge, Albert F Magnusen, Rudolf A Manz, Jörg Köhl, Christian M Karsten
B-1 cells constitute a unique subpopulation of lymphocytes residing mainly in body cavities like the peritoneal cavity (PerC) but are also found in spleen and bone marrow (BM). As innate-like B cells, they mediate first line immune defense through low-affinity natural IgM (nIgM) antibodies. PerC B-1 cells can egress to the spleen and differentiate into nIgM antibody-secreting plasma cells that recognize conserved exogenous and endogenous cellular structures. Homing to and homeostasis within the PerC are regulated by the chemokine CXCL13 released by PerC macrophages and stroma cells...
2018: Frontiers in Immunology
Amanda Crider, Tami Feng, Chirayu D Pandya, Talisha Davis, Ashwati Nair, Anthony Ahmed, Babak Baban, Gustavo Turecki, Anilkumar Pillai
Major depressive disorder (MDD) is one of the most common and debilitating neuropsychiatric illnesses. Accumulating evidence suggests a potential role of the immune system in the pathophysiology of MDD. The complement system represents one of the major effector mechanisms of the innate immune system, and plays a critical role in inflammation. However, the role of complement components in MDD is not well understood. Here, we found significant increase in component 3 (C3) expression in the prefrontal cortex (PFC) of depressed suicide subjects...
March 5, 2018: Brain, Behavior, and Immunity
David Chan-Rodriguez, Elsbeth L Walker
The micronutrient iron (Fe) is essential for photosynthesis, respiration, and many other processes, but it is only sparingly soluble in aqueous solution, making adequate acquisition by plants a serious challenge. Fe is a limiting factor for plant growth on approximately 30% of the world's arable lands. Moreover, Fe deficiency in humans is a global health issue, affecting 1.62 billion people, or about 25% of the world's population. It is imperative that we gain a better understanding of the mechanisms that plants use to regulate iron homeostasis, since these will be important targets for future biofortification and crop improvement strategies...
2018: Frontiers in Plant Science
Aric J Schultz, Vern B Carruthers
Egress is a crucial phase of the Toxoplasma gondii intracellular lytic cycle. This is a process that drives inflammation and is strongly associated with the pathogenesis observed during toxoplasmosis. Despite the link between this process and virulence, little is known about egress on a mechanistic or descriptive level. Previously published work has suggested that a phospholipase, lecithin-cholesterol acyltransferase (LCAT), secreted from the parasite's dense granules contributes to parasite growth, virulence, and egress...
January 2018: MSphere
Chenming Sun, Shi-You Chen
Systemic sclerosis (SSc) is a multisystem autoimmune disorder that is characterized by inflammation and fibrosis in the skin and internal organs. Previous studies indicate that inflammatory cells and cytokines play essential roles in the pathogenesis of SSc; however, the mechanisms that underlie the inflammation-driven development of SSc are not fully understood. In this study, we show that response gene to complement 32 (RGC32) is abundantly expressed in mouse macrophages in the early stage of bleomycin-induced SSc...
March 5, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Ayaka Ito, Chieko Sugita, Mizuho Ichinose, Yoshinobu Kato, Hiroshi Yamamoto, Toshiharu Shikanai, Mamoru Sugita
Pentatricopeptide repeat (PPR) proteins are known to play important roles in posttranscriptional regulation in plant organelles. However, the function of the majority of PPR proteins remains unknown. To examine their functions, Physcomitrella patens PpPPR_66 knockout (KO) mutants were generated and characterized. KO mosses exhibited a wild type-like growth phenotype but showed aberrant chlorophyll fluorescence due to defects in chloroplast NADH dehydrogenase-like (NDH) activity. Immuno-blot analysis suggested that disruption of PpPPR_66 led to a complete loss of the chloroplast NDH complex...
March 5, 2018: Plant Journal: for Cell and Molecular Biology
So Hee Dho, Jae Cheong Lim, Lark Kyun Kim
The complement is a part of the immune system that plays several roles in removing pathogens. Despite the importance of the complement system, the exact role of each component has been overlooked because the complement system was thought to be a nonspecific humoral immune mechanism that worked against pathogens. Decay-accelerating factor (DAF or CD55) is a known inhibitor of the complement system and has recently attracted substantial attention due to its role in various diseases, such as cancer, protein-losing enteropathy, and malaria...
February 2018: Immune Network
Christian Sina, Claudia Kemper, Stefanie Derer
The complement system is part of innate sensor and effector systems such as the Toll-like receptors (TLRs). It recognizes and quickly systemically and/or locally respond to microbial-associated molecular patterns (MAMPs) with a tailored defense reaction. MAMP recognition by intestinal epithelial cells (IECs) and appropriate immune responses are of major importance for the maintenance of intestinal barrier function. Enterocytes highly express various complement components that are suggested to be pivotal for proper IEC function...
February 24, 2018: Seminars in Immunology
Julie K Horton, Donna F Stefanick, Melike Çağlayan, Ming-Lang Zhao, Agnes K Janoshazi, Rajendra Prasad, Natalie R Gassman, Samuel H Wilson
Aprataxin (APTX) is a DNA-adenylate hydrolase that removes 5'-AMP blocking groups from abortive ligation repair intermediates. XRCC1, a multi-domain protein without catalytic activity, interacts with a number of known repair proteins including APTX, modulating and coordinating the various steps of DNA repair. CK2-phosphorylation of XRCC1 is thought to be crucial for its interaction with the FHA domain of APTX. In light of conflicting reports, the importance of XRCC1 phosphorylation and APTX function is not clear...
February 15, 2018: DNA Repair
Paola Concolino, Alessandra Costella
Congenital adrenal hyperplasia (CAH) comprises a group of autosomal recessive disorders caused by complete or partial defects in one of the several steroidogenic enzymes involved in the synthesis of cortisol from cholesterol in the adrenal glands. More than 95-99% of all cases of CAH are caused by deficiency of steroid 21-hydroxylase, an enzyme encoded by the CYP21A2 gene. Currently, CYP21A2 genotyping is considered a valuable complement to biochemical investigations in the diagnosis of 21-hydroxylase deficiency...
February 15, 2018: Molecular Diagnosis & Therapy
Vijay Jayaraman, Arpitha Suryavanshi, Pavithra Kalale, Jyothirmai Kunala, Hemalatha Balaram
Plasmodium falciparum (Pf), the causative agent of malaria, has an iron-sulfur cluster-containing class I fumarate hydratase (FH) that catalyzes the interconversion of fumarate to malate, a well-known reaction in the tricarboxylic acid cycle. In humans, the same reaction is catalyzed by class II FH that has no sequence or structural homology with the class I enzyme from Plasmodium. Fumarate is generated in large quantities in the parasite as a byproduct of AMP synthesis and is converted to malate by FH and then used in the generation of the key metabolites oxaloacetate, aspartate, and pyruvate...
February 15, 2018: Journal of Biological Chemistry
Xiao-Bing Cui, Jun-Na Luan, Kun Dong, Sisi Chen, Yongyi Wang, Wendy T Watford, Shi-You Chen
OBJECTIVE: The objective of this study is to determine the role and underlying mechanisms of RGC-32 (response gene to complement 32 protein) in atherogenesis. APPROACH AND RESULTS: RGC-32 was mainly expressed in endothelial cells of atherosclerotic lesions in both ApoE -/- (apolipoprotein E deficient) mice and human patients. RGC-32 deficiency (Rgc32 -/- ) attenuated the high-fat diet-induced and spontaneously developed atherosclerotic lesions in ApoE -/- mice without affecting serum cholesterol concentration...
February 15, 2018: Arteriosclerosis, Thrombosis, and Vascular Biology
Astrid J F Thielen, Iris M van Baarsen, Marlieke L Jongsma, Sacha Zeerleder, Robbert M Spaapen, Diana Wouters
BACKGROUND: To prevent unwanted complement activation and subsequent damage, complement activation must be tightly regulated on healthy host cells. Dysregulation of the complement system contributes to the pathology of diseases like Paroxysmal Nocturnal Hemoglobinuria and atypical Hemolytic Uremic Syndrome. To investigate complement regulator deficiencies, primary patient cells may be used, but access to patient cells may be limited and cells are heterogeneous between different patients...
February 12, 2018: Journal of Immunological Methods
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