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Complement deficiency

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https://www.readbyqxmd.com/read/28729648/a-haplotype-in-cfh-family-genes-confers-high-risk-of-rare-glomerular-nephropathies
#1
Yin Ding, Weiwei Zhao, Tao Zhang, Hao Qiang, Jianping Lu, Xin Su, Shuzhen Wen, Feng Xu, Mingchao Zhang, Haitao Zhang, Caihong Zeng, Zhihong Liu, Huimei Chen
Despite distinct renal lesions, a series of rare glomerular nephropathies are reportedly mediated by complement overactivation. Genetic variations in complement genes contribute to disease risk, but the relationship of genotype to phenotype has not been straightforward. Here, we screened 11 complement genes from 91 patients with atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G) and membranoproliferative glomerulonephritis type I (MPGN I), and identified the concomitant presence of three missense variations located within the human complement Factor H (CFH) gene cluster...
July 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28720814/small-rna-mediated-repression-of-subtilisin-production-in-bacillus-licheniformis
#2
Robert Hertel, Sandra Meyerjürgens, Birgit Voigt, Heiko Liesegang, Sonja Volland
The species Bacillus licheniformis includes important strains that are used in industrial production processes. Currently the physiological model used to adapt these processes is based on the closely related model organism B. subtilis. In this study we found that both organisms reveal significant differences in the regulation of subtilisin, their main natural protease and a product of industrial fermentation processes. We identified and characterized a novel antisense sRNA AprAs, which represents an RNA based repressor of apr, the gene encoding for the industrial relevant subtilisin protease...
July 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28718823/surface-chemistry-interactions-of-cationorm-with-films-by-human-meibum-and-tear-film-compounds
#3
Georgi As Georgiev, Norihiko Yokoi, Yana Nencheva, Nikola Peev, Philippe Daull
Cationorm(®) (CN) cationic nanoemulsion was demonstrated to enhance tear film (TF) stability in vivo possibly via effects on tear film lipid layer (TFLL). Therefore the interactions of CN with human meibum (MGS) and TFLL in vitro and in vivo deserve special study. MGS and CN were spread at the air/water interface of a Langmuir surface balance to ensure a range of MGS/CN oil phase ratios: 20/1, 10/1, 5/1, 3/1, 2/1 and 1/1. The films capability to reorganize during dynamic area changes was evaluated via the surface pressure-area compression isotherms and step/relaxation dilatational rheology studies...
July 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28717807/vitamin-d-status-and-prevalent-early-age-related-macular-degeneration-in-african-americans-and-caucasians-the-atherosclerosis-risk-in-communities-aric-study
#4
A E Millen, J Nie, M W Sahli, J A Mares, K J Meyers, B E K Klein, M J LaMonte, P L Lutsey, C A Andrews, R Klein
OBJECTIVES: Vitamin D status has been hypothesized to protect against development of early age-related macular degeneration (AMD) via its anti-inflammatory properties and its possible beneficial influence on blood pressure control. We investigated the association between vitamin D status and prevalent early AMD in a community-based cohort. DESIGN: This was a cross-sectional study. SETTING: This was a secondary data analysis of already existing data from the Atherosclerosis Risk in Communities Study (ARIC) cohort collected from 1990 to 1995...
2017: Journal of Nutrition, Health & Aging
https://www.readbyqxmd.com/read/28716421/dual-functioning-of-plant-arginases-provides-a-third-route-for-putrescine-synthesis
#5
Jigar Patel, Menaka Ariyaratne, Sheaza Ahmed, Lingxiao Ge, Vipaporn Phuntumart, Andrea Kalinoski, Paul F Morris
Two biosynthetic routes are known for putrescine, an essential plant metabolite. Ornithine decarboxylase (ODC) converts ornithine directly to putrescine, while a second route for putrescine biosynthesis utilizes arginine decarboxylase (ADC) to convert arginine to agmatine, and two additional enzymes, agmatine iminohydrolase (AIH) and N-carbamoyl putrescine aminohydrolase (NLP1) to complete this pathway. Here we show that plants can use ADC and arginase/agmatinase (ARGAH) as a third route for putrescine synthesis...
September 2017: Plant Science: An International Journal of Experimental Plant Biology
https://www.readbyqxmd.com/read/28715127/increased-aggression-and-lack-of-maternal-behavior-in-dio3-deficient-mice-are-associated-with-abnormalities-in-oxytocin-and-vasopressin-systems
#6
J Patrizia Stohn, M Elena Martinez, Maryam Zafer, Daniela López-Espíndola, Lauren M Keyes, Arturo Hernandez
Thyroid hormones regulate many aspects of brain development and function, and alterations in the levels of thyroid hormone action lead to abnormal anxiety- and depression-like behaviors. A complement of factors in the brain function independently of circulating levels of hormone to strictly controlled local thyroid hormone signaling. A critical factor is the type 3 deiodinase (DIO3), which is located in neurons and protects the brain from excessive thyroid hormone. Here we examined whether a local increase in brain thyroid hormone action secondary to DIO3 deficiency is of consequence for social behaviors...
July 17, 2017: Genes, Brain, and Behavior
https://www.readbyqxmd.com/read/28711717/phosphatidylinositol-4-phosphate-5-kinase-%C3%AE-contributes-to-toll-like-receptor-2-mediated-immune-responses-in-microglial-cells-stimulated-with-lipoteichoic-acid
#7
Tu Thi Ngoc Nguyen, Eunjeong Seo, Juyong Choi, Oanh Thi Tu Le, Ji Yun Kim, Ilo Jou, Sang Yoon Lee
Phosphatidylinositol 4,5-bisphosphate (PIP2) is an important lipid regulator of membrane signaling and remodeling processes. Accumulating evidence indicates a link between PIP2 metabolism and Toll-like receptor (TLR) signaling, a key transducer of immune responses such as inflammation, phagocytosis, and autophagy. Microglia are immune effector cells that serve as macrophages in the brain. Here, we examined the potential role of phosphatidylinositol 4-phosphate 5-kinase α (PIP5Kα), a PIP2-producing enzyme, in TLR2 signaling in microglial cells...
July 13, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/28711159/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-epidemiology-pathophysiology-and-clinics
#8
C Rafat, P Coppo, F Fakhouri, V Frémeaux-Bacchi, C Loirat, J Zuber, E Rondeau
Thrombotic microangiopathies (TMA) represent an eclectic group of conditions, which share hemolytic anemia and thrombocytopenia as a common defining basis. Remarkable breakthroughs in the physiopathological setting have allowed for a thorough recomposition of the disparate syndromes, which form the constellation of TMA. In this view, clinicians now discriminate thrombocytopenic thrombotic purpura (TTP) defined by a severe deficiency in ADAMTS13, which is rarely associated with a severe renal involvement and the hemolytic and uremic syndrome (HUS) in which renal impairment is the most prominent clinical feature...
July 12, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28711143/genomics-of-systemic-lupus-erythematosus-insights-gained-by-studying-monogenic-young-onset-systemic-lupus-erythematosus
#9
REVIEW
Linda T Hiraki, Earl D Silverman
Systemic lupus erythematosus (SLE) is a systemic, autoimmune, multisystem disease with a heterogeneous clinical phenotype. Genome-wide association studies have identified multiple susceptibility loci, but these explain a fraction of the estimated heritability. This is partly because within the broad spectrum of SLE are monogenic diseases that tend to cluster in patients with young age of onset, and in families. This article highlights insights into the pathogenesis of SLE provided by these monogenic diseases...
August 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/28710269/molecular-mechanism-and-genetic-determinants-of-buprofezin-degradation
#10
Xueting Chen, Junbin Ji, Leizhen Zhao, Jiguo Qiu, Chen Dai, Weiwu Wang, Jian He, Jiandong Jiang, Qing Hong, Xin Yan
Buprofezin is a widely used insect growth regulator whose residue has been frequently detected in the environment, posing a threat to aquatic organisms and non-target insects. Microorganisms play an important role in the degradation of buprofezin in the natural environment. However, the relevant catabolic pathway has not been fully characterized, and the molecular mechanism of catabolism is still completely unknown. Rhodococcus qingshengii YL-1 can utilize buprofezin as a sole source of carbon and energy for growth...
July 14, 2017: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/28706894/developmental-origins-and-nephron-endowment-in-hypertension
#11
REVIEW
Shari Gurusinghe, Anita Tambay, Christine B Sethna
Primary hypertension continues to be one of the main risk factors for cardiovascular disease worldwide. A stable intrauterine environment is critical for the future development and health of the fetus. The developing kidney has been found to be especially vulnerable during this time period, and epidemiological studies have demonstrated that an adverse in utero environment is associated with an increased risk of hypertension and chronic kidney disease. Macro- and micronutrient deficiencies as well as exposure to tobacco, alcohol, and certain medications during gestation have been shown to negatively impact nephrogenesis and reduce one's nephron number...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28694845/a-type-i-diacylglycerol-acyltransferase-modulates-triacylglycerol-biosynthesis-and-fatty-acid-composition-in-the-oleaginous-microalga-nannochloropsis-oceanica
#12
Hehong Wei, Ying Shi, Xiaonian Ma, Yufang Pan, Hanhua Hu, Yantao Li, Ming Luo, Henri Gerken, Jin Liu
BACKGROUND: Photosynthetic oleaginous microalgae are considered promising feedstocks for biofuels. The marine microalga, Nannochloropsis oceanica, has been attracting ever-increasing interest because of its fast growth, high triacylglycerol (TAG) content, and available genome sequence and genetic tools. Diacylglycerol acyltransferase (DGAT) catalyzes the last and committed step of TAG biosynthesis in the acyl-CoA-dependent pathway. Previous studies have identified 13 putative DGAT-encoding genes in the genome of N...
2017: Biotechnology for Biofuels
https://www.readbyqxmd.com/read/28694465/generation-of-complement-protein-c3-deficient-pigs-by-crispr-cas9-mediated-gene-targeting
#13
Wei Zhang, Guan Wang, Ying Wang, Yong Jin, Lihua Zhao, Qiang Xiong, Lining Zhang, Lisha Mou, Rongfeng Li, Haiyuan Yang, Yifan Dai
Complement protein C3 is the pivotal component of the complement system. Previous studies have demonstrated that C3 has implications in various human diseases and exerts profound functions under certain conditions. However, the delineation of pathological and physiological roles of C3 has been hampered by the insufficiency of suitable animal models. In the present study, we applied the clustered regularly interspaced short palindromic repeat (CRISPR)/CRISPR-associated (Cas) system to target the C3 gene in porcine fetal fibroblasts...
July 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28689072/nucleolytic-degradation-of-3-ending-overhangs-is-essential-for-dna-end-resection-in-reca-loading-deficient-recb-mutants-of-escherichia-coli
#14
Siniša Ivanković, Dušica Vujaklija, Damir Đermić
Degradation of a 5'-ending strand is the hallmark of the universal process of DNA double strand break (DSB) resection, which results in creation of the central recombination intermediate, a 3'-ending overhang. Here we show that in Escherichia coli recB1080/recB1067 mutants, which are devoid of RecBCD's nuclease and RecA loading activities, degradation of the unwound 3' tail is as essential as is degradation of its 5'-ending complement. Namely, a synergistic action of ExoI, ExoVII, SbcCD and ExoX single-strand specific exonucleases (ssExos) of 3'-5' polarity was essential for preserving cell viability, DNA repair and homologous recombination in the recB1080/recB1067 mutants, to the same extent as the redundant action of 5'-tail trimming ssExos RecJ and ExoVII...
June 27, 2017: DNA Repair
https://www.readbyqxmd.com/read/28684433/vacuolar-iron-transporter-tavit2-transports-fe-and-mn-and-is-effective-for-biofortification
#15
James M Connorton, Eleanor R Jones, Ildefonso Rodriguez-Ramiro, Susan Fairweather-Tait, Cristobal Uauy, Janneke Balk
Increasing the intrinsic nutritional quality of crops, known as biofortification, is viewed as a sustainable approach to alleviate micronutrient deficiencies. In particular iron deficiency anaemia is a major global health issue, but the iron content of staple crops such as wheat is difficult to change because of genetic complexity and homeostasis mechanisms. To identify target genes for biofortification of wheat (Triticum aestivum), we functionally characterized homologs of the Vacuolar Iron Transporter (VIT)...
July 6, 2017: Plant Physiology
https://www.readbyqxmd.com/read/28680821/a-unified-pathogenesis-for-kidney-diseases-including-genetic-diseases-and-cancers-by-the-protein-homeostasis-system-hypothesis
#16
REVIEW
Kyung-Yil Lee
Every cell of an organism is separated and protected by a cell membrane. It is proposed that harmony between intercellular communication and the health of an organism is controlled by a system, designated the protein-homeostasis-system (PHS). Kidneys consist of a variety of types of renal cells, each with its own characteristic cell-receptor interactions and producing characteristic proteins. A functional union of these renal cells can be determined by various renal function tests, and harmonious intercellular communication is essential for the healthy state of the host...
June 2017: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/28680352/analysis-of-changes-in-parathyroid-hormone-and-25-oh-vitamin-d-levels-with-respect-to-age-gender-and-season-a-data-mining-study
#17
Muhittin A Serdar, Başar Batu Can, Meltem Kilercik, Zeynep A Durer, Fehime Benli Aksungar, Mustafa Serteser, Abdurrahman Coskun, Aysel Ozpinar, Ibrahim Unsal
BACKGROUND: 25 (OH) vitamin D3 (25(OH)D) and parathyroid hormone (PTH) are important regulators of calcium homeostasis. The aim of this study was to retrospectively determine the cut-off for sufficient 25(OH)D in a four-season region and the influence of age, seasons, and gender on serum 25(OH)D and PTH levels. METHODS: Laboratory results of 9890 female and 2723 male individuals aged 38.8±22.1 years who had simultaneous measurements of 25(OH)D and PTH were retrospectively analyzed by statistical softwares...
January 2017: Journal of Medical Biochemistry
https://www.readbyqxmd.com/read/28674183/n-terminal-part-of-dishevelled-dep-domain-is-required-for-wnt-%C3%AE-catenin-signaling-in-mammalian-cells
#18
Petra Paclíková, Ondřej Bernatík, Tomasz Witold Radaszkiewicz, Vítězslav Bryja
Dishevelled (DVL) proteins are key mediators of Wnt/β-catenin signaling pathway. All DVL proteins contain three conserved domains -- DIX, PDZ and DEP. There is a consensus in the field that DIX domain is critical for Wnt/β-catenin signaling but contradictory evidence exists regarding the function of the DEP domain. It has been difficult until recently to test the importance of the DEP domain rigorously because of the interference with endogenous DVL, expressed in all Wnt-responsive cell lines. In this study, we took advantage of DVL KO (DVL1/DVL2/DVL3-triple knockout) cells, fully deficient in Wnt3a-induced signaling events, and performed series of rescue experiments...
July 3, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28671271/ndufa9-point-mutations-cause-a-variable-mitochondrial-complex-i-assembly-defect
#19
Fabian Baertling, Laura Sánchez-Caballero, Mariël Am van den Brand, Cheuk-Wing Fung, Sophelia Hoi-Shan Chan, Virginia Chun-Nei Wong, Debby M E Hellebrekers, Irenaeus F M de Coo, Jan Am Smeitink, Richard Jt Rodenburg, Leo Gj Nijtmans
Mitochondrial respiratory chain complex I consists of 44 different subunits and contains three functional modules: the Q-, the N- and the P-module. NDUFA9 is a Q-module subunit required for complex I assembly or stability. However, its role in complex I biogenesis has not been studied in patient fibroblasts. So far, a single patient carrying an NDUFA9 variant with a severe neonatally fatal phenotype has been reported. Via exome sequencing, we identified a novel homozygous NDUFA9 missense variant in another patient with a milder phenotype including childhood-onset progressive generalized dystonia and axonal peripheral neuropathy...
July 3, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28667799/reduced-bone-loss-in-a-murine-model-of-postmenopausal-osteoporosis-lacking-complement-component-3
#20
Danielle L MacKay, Thomas J Kean, Kristina G Bernardi, Heather S Haeberle, Catherine G Ambrose, Feng Lin, James E Dennis
The growing field of osteoimmunology seeks to unravel the complex interdependence of the skeletal and immune systems. Notably, we and others have demonstrated that complement signaling influences the differentiation of osteoblasts and osteoclasts, the two primary cell types responsible for maintaining bone homeostasis. However, the net effect of complement on bone homeostasis in vivo was unknown. Our published in vitro mechanistic work led us to hypothesize that absence of complement component 3 (C3), a central protein in the complement activation cascade, protects against bone loss in the ovariectomy-based model of postmenopausal osteoporosis...
July 1, 2017: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
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