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Hisashi Kamido, Shinya Yamamoto, Hideki Yokoi, Masashi Mizuno, Motoko Yanagita
C3 nephropathy is a renal disease caused by the aberrant activation of the alternative complement pathway. The long-term renal prognosis of C3 nephropathy is generally poor, and elucidation of its pathogenesis is clinically important. Genetic abnormalities within complement genes, encompassing autoantibodies targeting complement components and complement factor H-related proteins (CFHRs), can lead to abnormal complement activation. CFHR5 is one of the best-known responsible genes for C3 nephritis. Moreover, the renal prognosis can vary depending on the specific type of genetic mutation...
February 2024: Curēus
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JOURNAL ARTICLE
Benjamin Madden, Raman Deep Singh, Mark Haas, Lilian Mp Palma, Alok Sharma, Maria J Vargas, LouAnn Gross, Vivian Negron, Torell Nate, M Cristine Charlesworth, Jason D Theis, Samih H Nasr, Karl A Nath, Fernando C Fervenza, Sanjeev Sethi
C3 glomerulopathy (C3G) is a rare disease resulting from dysregulation of the alternative pathway of complement. C3G includes C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), both of which are characterized by bright glomerular C3 staining on immunofluorescence studies. However, on electron microscopy (EM), DDD is characterized by dense osmiophilic mesangial and intramembranous deposits along the glomerular basement membranes (GBM), while the deposits of C3GN are not dense. Why the deposits appear dense in DDD and not in C3GN is not known...
March 4, 2024: Kidney International
#3
JOURNAL ARTICLE
Ji-Hyun Lee, Seung Hoon Lee, Chanhyeok Jeon, Jinil Han, Sang-Hyon Kim, Jeehee Youn, Ye-Soo Park, Tae-Jong Kim, Jong-Seo Kim, Sungsin Jo, Tae-Hwan Kim, Chang-Nam Son
Ankylosing spondylitis (AS) is a chronic inflammatory disease, characterized by excessive new bone formation. We previously reported that the complement factor H-related protein-5 (CFHR5), a member of the human factor H protein family, is significantly elevated in patients with AS compared to other rheumatic diseases. However, the pathophysiological mechanism underlying new bone formation by CFHR5 is not fully understood. In this study, we revealed that CFHR5 and proinflammatory cytokines (TNF, IL-6, IL-17A, and IL-23) were elevated in the AS group compared to the HC group...
February 29, 2024: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
#4
JOURNAL ARTICLE
Zilac Espitaleta, Alex Domínguez-Vargas, Johanna Villamizar-Martínez, Martha Carrascal-Guzmán, Gustavo Guerrero-Tinoco, Diana Silva-Díaz, Richard Baquero, Claudia Pinto-Bernal, Luz González-Chaparro, Luisa Rojas-Rosas, Pilar Amado-Niño, Mariángel Castillo-Arteaga, Yeferson Alvarez-Gómez, Laura Arguello-Muñoz, William Morales-Camacho, Oscar León-Guerra, Eduardo Egea, Ricardo Galeano-Rodríguez, Ana Quintero-Gómez, Gustavo Aroca-Martínez, Carlos G Musso
Objectives. Atypical hemolytic uremic syndrome (aHUS) is a rare complement-mediated kidney disease with genetic predisposition and represents up to 10% of pediatric hemolytic uremic syndrome (HUS) cases. Few studies have evaluated aHUS in Latin American population. We studied a Colombian pediatric cohort to delineate disease presentation and outcomes. Methods. A multicenter cohort of 27 Colombian children with aHUS were included. Patients were grouped by age at onset. Clinical features were compared using analysis of variance (ANOVA) and Fisher exact tests...
2024: Global Pediatric Health
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JOURNAL ARTICLE
Yingzhou Shi, Hang Dong, Shiwei Sun, Xiaoqin Wu, Jiansong Fang, Jianbo Zhao, Junming Han, Zhongyue Li, Huixiao Wu, Luna Liu, Wanhong Wu, Yang Tian, Guandou Yuan, Xiude Fan, Chao Xu
BACKGROUNDS: To evaluate the causal correlation between complement components and non-viral liver diseases and their potential use as druggable targets. METHODS: We conducted Mendelian randomization (MR) to assess the causal role of circulating complements in the risk of non-viral liver diseases. A complement-centric protein interaction network was constructed to explore biological functions and identify potential therapeutic options. RESULTS: In the MR analysis, genetically predicted levels of complement C1q C chain (C1QC) were positively associated with the risk of autoimmune hepatitis (odds ratio [OR] 1...
December 7, 2023: Clinical and Molecular Hepatology
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JOURNAL ARTICLE
Ilhan E Acar, Tessel E Galesloot, Ulrich F O Luhmann, Sascha Fauser, Javier Gayán, Anneke I den Hollander, Everson Nogoceke
PURPOSE: To identify associations of common, low-frequency, and rare variants with advanced age-related macular degeneration (AMD) using whole genome sequencing (WGS). METHODS: WGS data were obtained for 2123 advanced AMD patients (participants of clinical trials for advanced AMD) and 2704 controls (participants of clinical trials for asthma [N = 2518] and Alzheimer's disease [N = 186]), and joint genotype calling was performed, followed by quality control of the dataset...
November 1, 2023: Investigative Ophthalmology & Visual Science
#7
JOURNAL ARTICLE
Sigridur Sunna Aradottir, Ann-Charlotte Kristoffersson, Erik Linnér, Diana Karpman
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) can be associated with mutations, deletions, or hybrid genes in factor H-related (FHR) proteins. METHODS: A child with aHUS was investigated. Genetics was assessed by Sanger and next generation sequencing. Serum FHR5 was evaluated by immunoblotting, ELISA, and by induction of rabbit red blood cell hemolysis in the presence/absence of recombinant human rFHR5. Mutagenesis was performed in HEK cells. RESULTS: A heterozygous genetic variant in factor H-related protein 5 (CFHR5), M514R, was found in the child, who also had a homozygous deletion of CFHR3/CFHR1, and antibodies to factor H, as well as low levels of C3...
November 13, 2023: Pediatric Nephrology
#8
JOURNAL ARTICLE
Bin Luo, Zujun Que, Xinyi Lu, Dan Qi, Zhi Qiao, Yun Yang, Fangfang Qian, Yi Jiang, Yan Li, Ronghu Ke, Xiaoyun Shen, Hua Xiao, Hegen Li, Erxi Wu, Jianhui Tian
BACKGROUND: Non-small cell lung cancer (NSCLC) remains a leading cause of cancer-related deaths worldwide, primarily due to its propensity for metastasis. Patients diagnosed with localized primary cancer have higher survival rates than those with metastasis. Thus, it is imperative to discover biomarkers for the early detection of NSCLC and the timely prediction of tumor metastasis to improve patient outcomes. METHODS: Here, we utilized an integrated approach to isolate and characterize plasma exosomes from NSCLC patients as well as healthy individuals...
November 13, 2023: Biological Procedures Online
#9
JOURNAL ARTICLE
Z-G Fei, K Zhen, F-J Zhang, P-Y Liu, H-Y Xu, H-H Chen
CFHR5 nephropathy is a type of clinical C3 glomerulopathy, which is a monogenic genetic disease caused by the internal replication of CFHR5 gene, a protein related to the complement regulatory factor H family. The disease seems to be prevalent only in people of Greek Cypriot descent. Because of the special variation of the internal replication of exon 2 and exon 3 of CFHR5 protein in the occurrence of disease, it has had a serious impact on local residents. At present, the mechanism of glomerular damage caused by CFHR5 protein mutations is still unclear...
October 2023: European Review for Medical and Pharmacological Sciences
#10
JOURNAL ARTICLE
Feng Xu, Changming Zhang, Mingchao Zhang, Xiaodong Zhu, Shuiqin Cheng, Zhen Cheng, Caihong Zeng, Song Jiang
BACKGROUND: Postinfectious glomerulonephritis with C3-dominant glomerular deposition (C3-PIGN) involves C3-dominant glomerular deposition without immunoglobulin. Atypical C3-PIGN involves persistent hypocomplementemia. We investigated the clinical features and explored complement-related gene mutations in atypical PIGN patients. METHODS: We enrolled atypical C3-PIGN patients and collected data regarding the clinical presentation and pathological characteristics and follow-up data...
October 17, 2023: International Urology and Nephrology
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JOURNAL ARTICLE
Alexandra E Butler, Abu Saleh Md Moin, Thozhukat Sathyapalan, Stephen L Atkin
INTRODUCTION: Upregulation of complement system factors are reported to be increased in polycystic ovary syndrome (PCOS) and may be due to obesity and insulin resistance rather than inherently due to PCOS. We directly compared complement factors from an obese, insulin-resistant PCOS population to a nonobese, non-insulin-resistant PCOS population in a proteomic analysis to investigate this. METHODS: Plasma was collected from 234 women (137 with PCOS and 97 controls) from a biobank cohort and compared to a nonobese, non-insulin-resistant population (24 with PCOS and 24 controls)...
August 4, 2023: Cells
#12
JOURNAL ARTICLE
Ximeng Chen, Jianan Wang, Jinyang Wang, Jingyun Ye, Ping Di, Chang Dong, Hong Lei, Chengbin Wang
BACKGROUND: Osteoarticular tuberculosis is one of the extrapulmonary tuberculosis (EPTB) diseases, which is mainly caused by infection of Mycobacterium tuberculosis (MTB) in bone and joints. The limitation of current clinical test methods is leading to a high misdiagnosis rate and affecting the treatment and prognosis. This study aims to search serum biomarkers that can assist in the diagnosis of osteoarticular tuberculosis. METHODS: Proteomics can serve as an important method in the discovery of disease biomarkers...
July 1, 2023: Clinica Chimica Acta; International Journal of Clinical Chemistry
#13
JOURNAL ARTICLE
Maria Jesus Iglesias, Laura Sanchez-Rivera, Manal Ibrahim-Kosta, Clément Naudin, Gaëlle Munsch, Louisa Goumidi, Maria Farm, Philip M Smith, Florian Thibord, Julia Barbara Kral-Pointner, Mun-Gwan Hong, Pierre Suchon, Marine Germain, Waltraud Schottmaier, Philip Dusart, Anne Boland, David Kotol, Fredrik Edfors, Mine Koprulu, Maik Pietzner, Claudia Langenberg, Scott M Damrauer, Andrew D Johnson, Derek M Klarin, Nicholas L Smith, David M Smadja, Margareta Holmström, Maria Magnusson, Angela Silveira, Mathias Uhlén, Thomas Renné, Angel Martinez-Perez, Joseph Emmerich, Jean-Francois Deleuze, Jovan Antovic, Jose Manuel Soria Fernandez, Alice Assinger, Jochen M Schwenk, Joan Carles Souto Andres, Pierre-Emmanuel Morange, Lynn Marie Butler, David-Alexandre Trégouët, Jacob Odeberg
Venous thromboembolism (VTE) is a common, multi-causal disease with potentially serious short- and long-term complications. In clinical practice, there is a need for improved plasma biomarker-based tools for VTE diagnosis and risk prediction. Here we show, using proteomics profiling to screen plasma from patients with suspected acute VTE, and several case-control studies for VTE, how Complement Factor H Related 5 protein (CFHR5), a regulator of the alternative pathway of complement activation, is a VTE-associated plasma biomarker...
June 7, 2023: Nature Communications
#14
JOURNAL ARTICLE
Priyanka Khandelwal, Sharan Thangaraju, Sriram Krishnamurthy, Alpana Ohri, Priya Pais, Georgie Mathew, Jyoti Sharma, Aditi Sharma, Pankaj Hari, Aditi Sinha, Geetika Singh, Arvind Bagga
BACKGROUND: Thrombotic microangiopathy (TMA) is usually caused due to dysregulation of the alternative complement pathway. Rarely, thrombotic microangiopathy is caused by non-complement mediated mutations in diacylglycerol kinase epsilon (DGKE); information about therapy and outcome of these patients is limited. METHODS: Medical records of patients, younger than 18 years, diagnosed with TMA and variants in DGKE were reviewed to include 12 patients from seven centers...
March 29, 2023: Pediatric Nephrology
#15
JOURNAL ARTICLE
Sara Gastoldi, Sistiana Aiello, Miriam Galbusera, Matteo Breno, Marta Alberti, Elena Bresin, Caterina Mele, Rossella Piras, Lucia Liguori, Donata Santarsiero, Ariela Benigni, Giuseppe Remuzzi, Marina Noris
INTRODUCTION: Comprehensive genetic analysis is essential to clinical care of patients with atypical haemolytic uremic syndrome (aHUS) to reinforce diagnosis, and to guide treatment. However, the characterization of complement gene variants remains challenging owing to the complexity of functional studies with mutant proteins. This study was designed: 1) To identify a tool for rapid functional determination of complement gene variants; 2) To uncover inherited complement dysregulation in aHUS patients who do not carry identified gene variants...
2023: Frontiers in Immunology
#16
JOURNAL ARTICLE
Yipei Hou, Li Wang, Chong Luo, Wenjing Tang, Rongxin Dai, Yunfei An, Xuemei Tang
OBJECTIVES: We sought to investigate the sex distribution, clinical presentations, disease outcomes and genetic background of early-onset paediatric systemic lupus erythematosus (eo-pSLE) in a single centre in China to help enable early diagnosis and timely treatment. METHODS: The clinical data of children aged less than five years with SLE (n = 19) from January 2012 to December 2021 were reviewed and analysed. We performed DNA sequencing in 11 out of 19 patients to survey the genetic etiologies...
February 22, 2023: Rheumatology
#17
JOURNAL ARTICLE
Alexandra E Butler, Abu Saleh Md Moin, Thozhukat Sathyapalan, Stephen L Atkin
Complement pathway proteins are reported to be increased in polycystic ovary syndrome (PCOS) and may be affected by obesity and insulin resistance. To investigate this, a proteomic analysis of the complement system was undertaken, including inhibitory proteins. In this cohort study, plasma was collected from 234 women (137 with PCOS and 97 controls). SOMALogic proteomic analysis was undertaken for the following complement system proteins: C1q, C1r, C2, C3, C3a, iC3b, C3b, C3d, C3adesArg, C4, C4a, C4b, C5, C5a, C5b-6 complex, C8, properdin, factor B, factor D, factor H, factor I, mannose-binding protein C (MBL), complement decay-accelerating factor (DAF) and complement factor H-related protein 5 (CFHR5)...
October 13, 2022: International Journal of Molecular Sciences
#18
JOURNAL ARTICLE
Wearn-Xin Yee, Christoph M Tang, Hayley Lavender
Neisseria meningitidis and Neisseria gonorrhoeae are important human pathogens that have evolved to bind the major negative regulator of the complement system, complement factor H (CFH). However, little is known about the interaction of pathogens with CFH-related proteins (CFHRs) which are structurally similar to CFH but lack the main complement regulatory domains found in CFH. Insights into the role of CFHRs have been hampered by a lack of specific reagents. We generated a panel of CFHR-specific monoclonal antibodies and demonstrated that CFHR5 was bound by both pathogenic Neisseria spp...
October 4, 2022: Infection and Immunity
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JOURNAL ARTICLE
Puxiu Shen, Jingcheng Yu, Xiaochuan Long, Xiankai Huang, Chao Tong, Xinzhuang Wang
Mastitis is a common disease of the dairy cattle, which affects the development of the dairy industry and leads to huge economic losses. Forsythoside A (FTA) has anti-inflammatory, antioxidant, antiviral and anti-apoptotic effects. However, the therapeutic effect and molecular mechanism of FTA on dairy cow mastitis remains unclear. In this study, bovine mammary epithelial cells (BMECs) were stimulated with lipoteichoic acid (LTA), a key virulence factor of Staphylococcus aureus (S. aureus), to construct in vitro models, and then treated with FTA...
September 21, 2022: Reproduction in Domestic Animals
#20
Sofia Menotti, Martino Donini, Giuseppina Pessolano, Livia Tiro, Maurizio Cantini, Jacopo Croce, Matteo Morandi, Filippo Mazzi, Katia Donadello, Oliviero Olivieri, Francesco Dima, Sergio De Marchi, Giovanni Gambaro, Enrico Polati, Lucia De Franceschi
No abstract text is available yet for this article.
November 2021: EJHaem
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