137 results on '"*PROPERDIN factor B"'
Search Results
2. Increased Alternative Complement Pathway Function and Improved Survival During Critical Illness.
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Bain, William, Li, Huihua, van der Geest, Rick, Moore, Sara R., Olonisakin, Tolani F., Ahn, Brian, Papke, Erin, Moghbeli, Kaveh, DeSensi, Rebecca, Rapport, Sarah, Saul, Melissa, Hulver, Mei, Zeyu Xiong, Mallampalli, Rama K., Ray, Prabir, Morris, Alison, Ma, Lina, Yohei Doi, Yingze Zhang, and Kitsios, Georgios D.
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CATASTROPHIC illness ,TRANSCRIPTION factors ,PROPERDIN factor B ,SEPSIS ,IMMUNE system - Abstract
The article presents a study related to an increased alternative complement pathway function and improved survival during critical illness. It mentions that patients with elevated alternative pathway (AH50) had an increased levels of activator protein (AP) factors B, H, and properdin, and some patients showed a "hyperinflammatory" subphenotype. It mentions overexuberant activation of complement system is thought to contribute to the pathogenesis of sepsis despite its role in immune function.
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- 2020
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3. The Challenges and Promise of Complement Therapeutics for Ocular Diseases.
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Park, Dong Ho, Connor, Kip M., and Lambris, John D.
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RETINAL degeneration ,OPHTHALMOLOGICAL therapeutics ,DISEASE progression ,INFLAMMATION ,PROPERDIN factor B - Abstract
Ocular inflammation is a defining feature of sight threating diseases and its dysregulation can catalyze and or propagate ocular neurodegenerative maladies such as age-related macular degeneration (AMD). The complement system, an intrinsic component of the innate immunity, has an integral role in maintaining immune-surveillance and homeostasis in the ocular microenvironment; however, overstimulation can drive ocular inflammatory diseases. The mechanism for complement disease propagation in AMD is not fully understood, although there is accumulating evidence showing that targeted modulation of complement-specific proteins has the potential to become a viable therapeutic approach. To date, a major focus of complement therapeutics has been on targeting the alternative complement system in AMD. Recent studies have outlined potential complement cascade inhibitors that might mitigate AMD disease progression. First-in-class complement inhibitors target the modulation of complement proteins C3, C5, factor B, factor D, and properdin. Herein, we will summarize ocular inflammation in the context of AMD disease progression, current clinical outcomes and complications of complement-mediated therapeutics. Given the need for additional therapeutic approaches for ocular inflammatory diseases, targeted complement modulation has emerged as a leading candidate for eliminating inflammation-driven ocular maladies. [ABSTRACT FROM AUTHOR]
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- 2019
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4. Looking back on the alternative complement pathway.
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Lachmann, Peter J.
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PROPERDIN factor B , *COMPLEMENT activation , *IMMUNOGLOBULINS , *BLOOD serum analysis , *CHEMICAL reactions - Abstract
The alternative pathway of complement originated from the Properdin pathway originally described by the Pillemer laboratory in the 1950s. This work generated great controversy and it took several decades for a consensus on its components, its reaction sequence and its functions to emerge. This paper reviews this history and attempts to clarify some of the ambiguities that remain. [ABSTRACT FROM AUTHOR]
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- 2018
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5. Polymorphisms in genes related to the complement system and antibody-mediated cardiac allograft rejection.
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Marrón-Liñares, Grecia M., Núñez, Lucía, Crespo-Leiro, María G., Barge-Caballero, Eduardo, Pombo, Jorge, Paniagua-Martin, María Jesús, Suarez-Fuentetaja, Natalia, Cid, Javier, Grille-Cancela, Zulaika, Muñiz-Garcia, Javier, Tan, Carmela D., Rodríguez, E. Rene, Vázquez-Rodríguez, José Manuel, and Hermida-Prieto, Manuel
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HEART transplantation , *GENETIC polymorphisms , *HOMOGRAFTS , *IMMUNOGLOBULINS , *PROPERDIN factor B - Abstract
Background Heart transplantation (HT) is a life-saving treatment for patients with end-stage heart failure. One of the main problems after HT is the humoral response termed antibody-mediated rejection (AMR). Complement activation plays a key role in AMR contributing to graft damage. The aim of this study was to analyze genetic variants in genes related to the complement pathways that could be associated with the development of AMR. Methods Analysis of 51 genes related to the complement pathway was performed by next-generation sequencing in 46 HT recipients, 23 with and 23 without AMR. Statistical analysis was performed with SNPstats and R. Results We identified 2 single nucleotide polymorphisms, 1 in the mannose-binding lectin 2 gene (p.Gly54Asp- MBL2 ) and 1 in the complement factor properdin gene (p.Asn428(p=)- CFP ), that showed significant association with the absence and development of AMR, respectively. Moreover, the presence of the rare allele in p.Gly54Asp- MBL2 control patients correlated with an immunodeficiency of mannose-binding lectin (6.24 ng/ml vs 207.50 ng/ml, p < 0.01), whereas the presence of the rare allele p.Asn428(p=)- CFP in patients with AMR correlated with higher levels of properdin protein (14.65 μg/ml vs 10.77 μg/ml, p < 0.05). Conclusions AMR is a complex phenotype affected by many recipient factors. Variants in p.Gly54Asp- MBL2 and p.Asn428(p=)- CFP genes, encoding mannose-binding lectin 2 and properdin, may influence the risk of AMR. [ABSTRACT FROM AUTHOR]
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- 2018
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6. The properdin pathway: an “alternative activation pathway” or a “critical amplification loop” for C3 and C5 activation?
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Harrison, Richard A.
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PROPERDIN factor B , *COMPLEMENT activation , *ENZYME activation , *IMMUNITY , *WAVE amplification - Abstract
This review is not intended to cover in detail all aspects of the discovery and evolution of our understanding of the “alternative pathway” of complement activation, there are many excellent reviews that do this (see Fearon (CRC Crit Rev Immunol 1:1–32,
1979 ), Pangburn and Müller-Eberhard (Springer Semin Immunopathol 7:163–192,1984 )), but instead to give sufficient background for current concepts to be put in context. The prevailing textbook view, of components having a primary role as an alternative “pathway” for C3 activation, is challenged, with an argument developed for the primary role of the system being that of providing a surface-dependent amplification loop for both C3 and C5 activation. Whatever the mechanism by which the initial C3b molecule is generated, deposition onto a surface has the potential to target that surface for elimination. Elimination or escape from initial targeting is determined by a sophisticated and highly regulated amplification loop for C3 activation. This viewpoint of the system is then briefly developed to provide a context for therapeutic treatment of disease caused, at least in part, by dysregulated amplification of C3 activation, and to highlight some of the challenges that such therapies will face and need to address. [ABSTRACT FROM AUTHOR]- Published
- 2018
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7. Complement’s hidden arsenal: New insights and novel functions inside the cell.
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Liszewski, M. Kathryn, Elvington, Michelle, Kulkarni, Hrishikesh S., and Atkinson, John P.
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COMPLEMENT (Immunology) , *NATURAL immunity , *HOSTS (Biology) , *CELL metabolism , *PROPERDIN factor B , *T cells - Abstract
A key component of both innate and adaptive immunity, new understandings of the complement system are expanding its roles beyond that traditionally appreciated. Evidence is accumulating that complement has an intracellular arsenal of components that provide not only immune defense, but also assist in key interactions for host cell functions. Although early work has primarily centered on T cells, the intracellular complement system likely functions in many if not most cells of the body. Some of these functions may trace their origins to the primitive complement system that began as a primeval form of C3 likely tasked for protection from intracellular pathogen invasion. This later expanded to include extracellular defense as C3 became a secreted protein to patrol the vasculature. Other components were added to the growing system including regulators to protect host cells from the indiscriminate effects of this potent system. Contemporary cells may retain some of these vestigial remnants. We now know that a) C3 serves as a damage-associated molecular pattern (in particular by coating pathogens that translocate into cells), b) most cells store C3 and recycle C3(H 2 O) for immediate use, and c) C3 assists in cellular survival and metabolic reprogramming. Other components also are part of this hidden arsenal including C5, properdin, factors H and B, and complement receptors. Importantly, better definition of the intracellular complement system may translate into new target discovery to assist in creating the next generation of complement therapeutics. [ABSTRACT FROM AUTHOR]
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- 2017
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8. Properdin binding to complement activating surfaces depends on initial C3b deposition.
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Harboe, Morten, Johnson, Christina, Nymo, Stig, Ekholt, Karin, Schjalm, Camilla, Lindstad, Julie K., Pharo, Anne, Hellerud, Bernt Christian, Ekdahl, Kristina Nilsson, Mollnes, Tom Eirik, and Nilsson, Per H.
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PROPERDIN factor B , *PATTERN recognition systems , *ESCHERICHIA coli , *MYELOPEROXIDASE , *FLOW cytometry - Abstract
Two functions have been assigned to properdin; stabilization of the alternative convertase, C3bBb, is well accepted, whereas the role of properdin as pattern recognition molecule is controversial. The presence of nonphysiological aggregates in purified properdin preparations and experimental models that do not allow discrimination between the initial binding of properdin and binding secondary to C3b deposition is a critical factor contributing to this controversy. In previous work, by inhibiting C3, we showed that properdin binding to zymosan and Escherichia coli is not a primary event, but rather is solely dependent on initial C3 deposition. In the present study, we found that properdin in human serum bound dose-dependently to solid-phase myeloperoxidase. This binding was dependent on C3 activation, as demonstrated by the lack of binding in human serum with the C3-inhibitor compstatin Cp40, in C3-depleted human serum, or when purified properdin is applied in buffer. Similarly, binding of properdin to the surface of human umbilical vein endothelial cells or Neisseria meningitidis after incubation with human serum was completely C3-dependent, as detected by flow cytometry. Properdin, which lacks the structural homology shared by other complement pattern recognition molecules and has its major function in stabilizing the C3bBb convertase, was found to bind both exogenous and endogenousmolecular patterns in a completely C3-dependent manner. We therefore challenge the view of properdin as a pattern recognition molecule, and argue that the experimental conditions used to test this hypothesis should be carefully considered, with emphasis on controlling initial C3 activation under physiological conditions. [ABSTRACT FROM AUTHOR]
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- 2017
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9. Monomeric C-reactive protein inhibits renal cell-directed complement activation mediated by properdin.
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O'Flynn, Joseph, van der Pol, Pieter, Dixon, Karen O., Prohászka, Zoltán, Daha, Mohamed R., and van Kooten, Cees
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C-reactive protein , *PROPERDIN factor B , *DIAGNOSTIC use of flow cytometry - Abstract
Previous studies have shown that complement activation on renal tubular cells is involved in the induction of interstitial fibrosis and cellular injury. Evidence suggests that the tubular cell damage is initiated by the alternative pathway (AP) of complement with properdin having an instrumental role. Properdin is a positive regulator of the AP, which can bind necrotic cells as well as viable proximal tubular epithelial cells (PTECs), inducing complement activation. Various studies have indicated that in the circulation there is an unidentified inhibitor of properdin. We investigated the ability of C-reactive protein (CRP), both in its monomeric (mCRP) and pentameric (pCRP) form, to inhibit AP activation and injury in vitro on renal tubular cells by fluorescent microscopy, ELISA, and flow cytometry. We demonstrated that preincubation of properdin with normal human serum inhibits properdin binding to viable PTECs. We identified mCRP as a factor able to bind to properdin in solution, thereby inhibiting its binding to PTECs. In contrast, pCRP exhibited no such binding and inhibitory effect. Furthermore, mCRP was able to inhibit properdindirected C3 and C5b-9 deposition on viable PTECs. The inhibitory ability of mCRP was not unique for viable cells but also demonstrated for binding to necrotic Jurkat cells, a target for properdin binding and complement activation. In summary, mCRP is an inhibitor of properdin in both binding to necrotic cells and viable renal cells, regulating complement activation on the cell surface. We propose that mCRP limits amplification of tissue injury by controlling properdin-directed complement activation by damaged tissue and cells. [ABSTRACT FROM AUTHOR]
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- 2016
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10. Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy.
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Corvillo, F., Bravo García‐Morato, M., Nozal, P., Garrido, S., Tortajada, A., Rodríguez de Córdoba, S., and López‐Trascasa, M.
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PROPERDIN factor B , *BIOMARKERS , *GLOMERULONEPHRITIS , *PROPROTEIN convertases , *SERUM , *NEISSERIA infections , *AUTOANTIBODIES - Abstract
Properdin (P) stabilizes the alternative pathway (AP) convertases, being the only known positive regulator of the complement system. In addition, P is a pattern recognition molecule able to initiate directly the AP on non-self surfaces. Although P deficiencies have long been known to be associated with Neisseria infections and P is often found deposited at sites of AP activation and tissue injury, the potential role of P in the pathogenesis of complement dysregulation-associated disorders has not been studied extensively. Serum P levels were measured in 49 patients with histological and clinical evidence of C3 glomerulopathy (C3G). Patients were divided into two groups according to the presence or absence of C3 nephritic factor (C3NeF), an autoantibody that stabilizes the AP C3 convertase. The presence of this autoantibody results in a significant reduction in circulating C3 ( P < 0·001) and C5 levels ( P < 0·05), but does not alter factor B, P and sC5b-9 levels. Interestingly, in our cohort, serum P levels were low in 17 of the 32 C3NeF-negative patients. This group exhibited significant reduction of C3 ( P < 0·001) and C5 ( P < 0·001) and increase of sC5b-9 ( P < 0·001) plasma levels compared to the control group. Also, P consumption was correlated significantly with C3 ( r = 0·798, P = 0·0001), C5 ( r = 0·806, P < 0·0001), sC5b-9 ( r = −0·683, P = 0·043) and a higher degree of proteinuria ( r = −0·862, P = 0·013). These results illustrate further the heterogeneity among C3G patients and suggest that P serum levels could be a reliable clinical biomarker to identify patients with underlying surface AP C5 convertase dysregulation. [ABSTRACT FROM AUTHOR]
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- 2016
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11. Properdin Provides Protection from Citrobacter rodentium- Induced Intestinal Inflammation in a C5a/IL-6-Dependent Manner.
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Jain, Umang, Qi Cao, Thomas, Nikhil A., Woodruff, Trent M., Sehwaeble, Wilhelm J., Stover, Cordula M., and Stadnyk, Andrew W.
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CITROBACTER , *PROPERDIN factor B , *INTESTINAL diseases , *COMPLEMENT (Immunology) , *EPITHELIAL cells , *GENETICS - Abstract
Citrobacter rodentium is an attaching and effacing mouse pathogen that models enteropathogenic and enterohemorrhagic Escherichia coli in humans. The complement system is an important innate defense mechanism; however, only scant information is available about the role of complement proteins during enteric infections. In this study, we examined the impact of the lack of properdin, a positive regulator of complement, in C. rodentium-induced colitis. Following infection, properdin knockout (PKO) mice had increased diarrhea and exacerbated inflammation combined with defective epithelial cell-derived IL-6 and greater numbers of colonizing bacteria. The defect in the mucosal response was reversed by administering exogenous properdin to PKO mice. Then, using in vitro and in vivo approaches, we show that the mechanism behind the exacerbated inflammation of PKO mice is due to a failure to increase local C5a levels. We show that C5a directly stimulates IL-6 production from colonic epithelial cells and that inhibiting C5a in infected wild-type mice resulted in defective epithelial IL-6 production and exacerbated inflammation. These outcomes position properdin early in the response to an infectious challenge in the colon, leading to complement activation and C5a, which in turn provides protection through IL-6 expression by the epithelium. Our results unveil a previously unappreciated mechanism of intestinal homeostasis involving complement, C5a, and IL-6 during bacteria-triggered epithelial injury. [ABSTRACT FROM AUTHOR]
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- 2015
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12. Differential Complement Activation Pathways Promote C3b Deposition on Native and Acetylated LDL thereby Inducing Lipoprotein Binding to the Complement Receptor 1.
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Klop, Boudewijn, van der Pol, Pieter, van Bruggen, Robin, Yanan Wang, de Vries, Marijke A., van Santen, Selvetta, O'Flynn, Joseph, van de Geij, Gert-Jan M., Njo, Tjin L., Janssen, Hans W., de Man, Peter, Jukema, J. Wouter, Rabelink, Ton J., Rensen, Patrick C. N., van Kooten, Cees, and Castro Cabezas, Manuel
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LIPOPROTEINS , *LOW density lipoproteins , *LECTINS , *PROPERDIN factor B , *CARRIER proteins - Abstract
Lipoproteins can induce complement activation resulting in opsonization and binding of these complexes to complement receptors. We investigated the binding of opsonized native LDL and acetylated LDL (acLDL) to the complement receptor 1 (CR1). Binding of complement factors C3b, IgM, C1q, mannose-binding lectin (MBL), and properdin to LDL and acLDL were investigated by ELISA. Subsequent binding of opsonized LDL and acLDL to CR1 on CR1-transfected Chinese Hamster Ovarian cells (CHO-CR1) was tested by flow cytometry. Both native LDL and acLDL induced complement activation with subsequent C3b opsonization upon incubation with normal human serum. Opsonized LDL and acLDL bound to CR1. Binding to CHO-CR1 was reduced by EDTA, whereas MgEGTA only reduced the binding of opsonized LDL, but not of acLDL suggesting involvement of the alternative pathway in the binding of acLDL to CR1. In vitro incubations showed that LDL bound C1q, whereas acLDL bound to C1q, IgM, and properdin. MBL did neither bind to LDL nor to acLDL. The relevance of these findings was demonstrated by the fact that ex vivo up-regulation of CR1 on leukocytes was accompanied by a concomitant increased binding of apolipoprotein B-containing lipoproteins to leukocytes without changes in LDL-receptor expression. In conclusion, CR1 is able to bind opsonized native LDL and acLDL. Binding of LDL to CR1 is mediated via the classical pathway, whereas binding of acLDL is mediated via both the classical and alternative pathways. Binding of lipoproteins to CR1 may be of clinical relevance due to the ubiquitous cellular distribution of CR1. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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13. Sistema del complemento e inmunocomplejos circulantes en el mieloma múltiple Complement system and circulating immune complexes in multiple myeloma
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Carlos Hernández Padrón, Julio Fernández Águila, Rinaldo Villaescusa Blanco, Edgardo Espinosa Martínez, Rafael Losada Buchillón, José R Mesa Cuervo, and Alelí Plasencia Ternblón
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MIELOMA MULTIPLE ,VIA CLASICA DEL COMPLEMENTO ,VIA ALTERNATIVA DEL COMPLEMENTO ,PRO-PERDINA FACTOR B ,COMPLEMENTO 3 ,COMPLEMENTO 4 ,COMPLEJO ANTIGENO-ANTICUERPO ,MULTIPLE MYELOMA ,COMPLEMENT PATHWAY ,CLASSICAL ,ALTERNATIVE ,PROPERDIN FACTOR B ,COMPLEMENT 3 ,COMPLEMENT 4 ,ANTIGEN-ANTIBODY COMPLEX ,Diseases of the blood and blood-forming organs ,RC633-647.5 ,Immunologic diseases. Allergy ,RC581-607 - Abstract
Se estudiaron 39 enfermos con mieloma múltiple (12 IgA y 27 IgG) en diferentes estadios de la enfermedad: 9 en estadios I + II y 30 en el estadio III, y en diferentes fases clínicas: 17 en fase activa precoz, 7 en fase estable, 8 en recaída y 7 en fase refractaria. Se evaluó la actividad hemolítica de la vía clásica, de la vía alterna y del factor B del complemento. Se cuantificaron los componentes C3 y C4 y se determinó la presencia de inmunocomplejos circulantes. Se comprobó una disminución significativa de la actividad hemolítica de la vía alterna (p= 0,0002), de la actividad hemolítica del factor B (p= 0,00001) y de los niveles séricos de C3 (p= 0,00001). Estas alteraciones se mantuvieron independientemente del tipo de paraproteína, estadio y fase clínica de los pacientes estudiados39 patients with multiple myeloma (12 IgA and 27 IgG) in different stages of the diseases were studied. 9 were in stages I + II and 30 in stage III. As regards the clinical phases, 17 were in active early phase, 7 in steady phase, 8 in relapse and 7 in refractory phase. The hemolytic activity of the classical pathway, of the alternative pathway and of the complement factor B was evaluated. Components C3 and C4 were quantitated and the presence of circulating immune complexes was determined. A significant reduction of the hemolytic activity of the alternative pathway (p=0.0002), of the hemolytic activity of factor B (p=0.00001), and of the serum levels of C3 (p=0.00001) was proved. These alterations occurred regardless of the type of paraprotein and of the stage and cinical phase of the studied patients
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- 1999
14. Properdin has an ascendancy over factor H regulation in complement-mediated renal tubular damage.
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Seiji Nagamachi, Isao Ohsawa, Hiyori Suzuki, Nobuyuki Sato, Hiroyuki Inoshita, Atsuko Hisada, Daisuke Honda, Mamiko Shimamoto, Yoshio Shimizu, Satoshi Horikoshi, and Yasuhiko Tomino
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PROPERDIN factor B ,PROTEINURIA ,KIDNEY diseases ,KIDNEY tubules ,EPITHELIAL cells ,MULTIVARIATE analysis ,PROTEIN expression ,DISEASES - Abstract
Background Urinary (U)-complement components have been detected in patients with proteinuric renal diseases, and complement activation via the alternative pathway (AP) is believed to play a role in renal tubular damage. The present study aimed to examine the regulation of complement AP activation in patients with renal tubular damage by focusing on the balance between properdin (P) and factor H (fH). Methods In the in vivo studies, U concentrations of P, fH and membrane attack complex (MAC) were measured in patients with renal diseases using an enzyme-linked immunosorbent assay (ELISA), and their relationships with the clinical data were evaluated. In the in vitro studies, human proximal tubular epithelial cells (PTECs) were incubated with normal human serum (NHS), P-depleted serum (PDS), purified P and/or fH. Changes in cell morphology and phenotype were assessed by microscopy, real-time polymerase chain reaction (PCR), immunostaining and a cell viability assay. Results The U-P, fH and MAC concentrations were significantly higher in patients with renal disease than in normal controls and correlated with the U-protein and tubular damage markers. Furthermore, multivariate analysis revealed a relationship between P levels and tubular damage markers. There were no significant changes in morphology and mRNA expression in the AP components (P, fH, fB, C3, C5 and C9) after the addition of up to 25% NHS. Dosedependent depositions of P or fH were observed after the addition of P or fH on PTECs. Depositions of P were not inhibited by fH in a mixture of a fixed concentration of P and a variable concentration of fH, and vice versa. Preincubation with the fixed concentration of P before the addition of NHS or PDS increased the depositions of P, C3 and MAC compared with incubation with intact NHS or intact PDS only; the depositions of C3 and MAC showed a serum-dependent trend. Preincubation with P before NHS addition significantly suppressed cell viability without causing morphological changes. Conclusions In the pathogenesis of renal tubular damage, P can directly bind to PTECs and may accelerate AP activation by surpassing fH regulation. [ABSTRACT FROM AUTHOR]
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- 2014
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15. Low-dose recombinant properdin provides substantial protection against Streptococcus pneumoniae and Neisseria meningitidis infection.
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Youssif Mohammed Ali, Hayat, Azam, Saeed, Bayad Mawlood, Haleem, Kashif S., Alshamrani, Saleh, Kenawy, Hany I., Ferreira, Viviana P., Saggu, Gurpanna, Buchberger, Anna, Lachmann, Peter J., Sim, Robert B., Goundis, Dimitrios, Andrew, Peter W., Lynch, Nicholas J., and Schwaeble, Wilhelm J.
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PROPERDIN factor B , *STREPTOCOCCUS pneumoniae , *NEISSERIA meningitidis , *INFECTION , *IMMUNOTHERAPY - Abstract
Modern medicine has established three central antimicrobial therapeutic concepts: vaccination, antibiotics, and, recently, the use of active immunotherapy to enhance the immune response toward specific pathogens. The efficacy of vaccination and antibiotics is limited by the emergence of new pathogen strains and the increased incidence of antibiotic resistance. To date, immunotherapy development has focused mainly on cytokines. Here we report the successful therapeutic application of a complement component, a recombinant form of properdin (Pn), with significantly higher activity than native properdin, which promotes complement activation via the alternative pathway, affording protection against N. menigitidis and S. pneumoniae. In a mouse model of infection, we challenged C57BL/6 WT mice with N. menigitidis B-MC58 6 h after i.p. administration of Pn (100 ug/mouse) or buffer alone. Twelve hours later, all control mice showed clear symptoms of infectious disease while the Pn treated group looked healthy. After 16 hours, all control mice developed sepsis and had to be culled, while only 10% of Pn treated mice presented with sepsis and recoverable levels of live Meningococci. In a parallel experiment, mice were challenged intranasally with a lethal dose of S. pneumoniae D39. Mice that received a single i.p. dose of Pn at the time of infection showed no signs of bacteremia at 12 h postinfection and had prolonged survival times compared with the saline-treated control group (P < 0.0001). Our findings show a significant therapeutic benefit of Pn administration and suggest that its antimicrobial activity could open new avenues for fighting infections caused by multidrug-resistant neisserial or streptococcal strains. [ABSTRACT FROM AUTHOR]
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- 2014
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16. Expression and functional analysis of properdin in zebrafish Danio rerio.
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Zhang, Yanjie, Chen, Jiayan, Yao, Feng, Ji, Dongrui, Li, Hongyan, and Zhang, Shicui
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ZEBRA danio , *GENE expression in fishes , *PROPERDIN factor B , *STRUCTURE-activity relationships , *FUNCTIONAL analysis , *FISH larvae , *IMMUNE response , *FISH immunology - Abstract
Abstract: Properdin, an upregulator of the alternative complement pathway, has been thoroughly studied in the mammalian species, but its research in the lower vertebrates such as fish is rather limited. Additionally, information regarding the structure–activity relationship of properdin remains rather fragmentary. In this report, we showed that zebrafish properdin gene zfp was abundantly expressed in the liver of adult fish, while it was primarily expressed in the brain, neural plate, developing lens, and neutrophil in the early embryos/larvae. Recombinant TSR modules of zfP were demonstrated to be able to bind to C3b, LPS, LTA and both gram-negative and positive bacteria. Moreover, TSR5 of zfP was able to enhance the phagocytosis of microbes by macrophages. These results together support the notion that properdin is a pattern recognition molecule capable of identifying non-self antigens/structures, and indicate that TSR5 plays a central role in the capacity of properdin to promote phagocytosis. It is also suggested that properdin is associated with the pattern formation and immune defense of early developing embryos/larvae. [Copyright &y& Elsevier]
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- 2013
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17. Aurin tricarboxylic acid self-protects by inhibiting aberrant complement activation at the C3 convertase and C9 binding stages
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Lee, Moonhee, Guo, Jian-Ping, McGeer, Edith G., and McGeer, Patrick L.
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TRICARBOXYLIC acids , *COMPLEMENT activation , *DEGENERATION (Pathology) , *PROPERDIN factor B , *CARBOXYLIC acids , *RHEUMATOID arthritis - Abstract
Abstract: Aberrant complement activation is known to exacerbate the pathology in a spectrum of degenerative diseases of aging. We previously reported that aurin tricarboxylic acid (ATA) is an orally effective agent which prevents formation of the membrane attack complex of complement. It inhibits C9 attachment to tissue bound C5b678 and thus prevents bystander lysis of host cells. In this study, we investigated the effects of ATA on the alternative complement pathway. We found that ATA prevented cleavage of the tissue bound properdin-C3b-Factor B complex into the active C3 convertase enzyme properdin-C3b-Factor Bb. This inhibition was reversed by adding Factor D to the serum. Using enzyme-linked immunosorbent type assays, we established that ATA binds directly to Factor D and C9 but not to properdin or other complement proteins. We conclude that ATA, by inhibiting at two stages of the alternative pathway, might be a particularly effective therapeutic agent in conditions such as macular degeneration, paroxysmal nocturnal hemoglobinemia, and rheumatoid arthritis, in which activation of the alternative complement pathway initiates self damage. [Copyright &y& Elsevier]
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- 2013
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18. Properdin and factor H: opposing players on the alternative complement pathway "see-saw".
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Kouser, Lubna, Abdul-Aziz, Munirah, Nayak, Annapurna, Stover, Cordula M., Sim, Robert B., and Kishore, Uday
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PROPERDIN factor B ,KERATINOCYTES ,FIBROBLASTS ,NEUTROPHILS ,IMMUNOLOGY - Abstract
Properdin and factor H are two key regulatory proteins having opposite functions in the alternative complement pathway. Properdin up-regulates the alternative pathway by stabilizing the C3bBb complex, whereas factor H downregulates the pathway by promoting proteolytic degradation of C3b. While factor H is mainly produced in the liver, there are several extrahepatic sources. In addition to the liver, factor H is also synthesized in fetal tubuli, keratinocytes, skin fibroblasts, ocular tissue, adipose tissue, brain, lungs, heart, spleen, pancreas, kidney, muscle, and placenta. Neutrophils are the major source of properdin, and it is also produced by monocytes, T cells and bone marrow progenitor cell line. Properdin is released by neutrophils from intracellular stores following stimulation by N-formyl-methionine-leucine-phenylalanine (fMLP) and tumor necrosis factor alpha (TNF-α). The HEP G2 cells derived from human liver has been found to produce functional properdin. Endothelial cells also produce properdin when induced by shear stress, thus is a physiological source for plasma properdin. The diverse range of extrahepatic sites for synthesis of these two complement regulators suggests the importance and need for local availability of the proteins. Here, we discuss the significance of the local synthesis of properdin and factor H. This assumes greater importance in view of recently identified unexpected and novel roles of properdin and factor H that are potentially independent of their involvement in complement regulation. [ABSTRACT FROM AUTHOR]
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- 2013
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19. Circulating complement activation in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.
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Gou, Shen-Ju, Yuan, Jun, Chen, Min, Yu, Feng, and Zhao, Ming-Hui
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NEUTROPHILS , *CYTOPLASM , *VASCULITIS , *ENZYME-linked immunosorbent assay , *PROPERDIN factor B - Abstract
Studies in animal models suggest that complement activation is crucial in the pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Here we investigate the circulating complement activation profile of 66 patients with active stage AAV compared to that of 54 patients with AAV in remission. Plasma levels of C3a, C5a, soluble C5b-9, and Bb, all determined by enzyme-linked immunosorbent assay, were significantly higher in active stage than in remission of AAV, while plasma levels of properdin were significantly lower in the former than the latter disease stage. There was no significant difference in the plasma levels of C4d between active stage and remission. The plasma level of Bb in patients with active AAV significantly correlated with the proportion of total and cellular crescents in the renal biopsy, the erythrocyte sedimentation rate, and the Birmingham Vasculitis Activity Scores. Thus, systemic activation of complement by the alternative pathway takes place in human AAV. Circulating Bb might be a useful biomarker in assessing disease activity of AAV. [ABSTRACT FROM AUTHOR]
- Published
- 2013
- Full Text
- View/download PDF
20. Local release of properdin in the cellular microenvironment: Role in pattern recognition and amplification of the alternative pathway of complement.
- Author
-
Cortes, Claudio, Ohtola, Jennifer A., Saggu, Gurpanna, and Ferreira, Viviana Patricia
- Subjects
PROPERDIN factor B ,PROTEINS ,OLIGOMERS ,MICROORGANISMS ,NEUTROPHILS - Abstract
Properdin, the only positive regulatory protein of the complement system, acts as both a stabilizer of the alternative pathway convertases and as a selective pattern recognition molecule of certain microorganisms and host cells (i.e. apoptotic/necrotic cells) by serving as a platform for de novo C3b,Bb assembly. Properdin, a highly positively charged protein, normally exists as cyclic dimers (P2), trimers (P3), and tetramers (P4) of head-to-tail associations of monomeric 53kDa subunits. While most complement proteins are produced mainly in the liver, properdin is synthesized primarily by various cell types including neutrophils, monocytes, primary T cells, and shear-stressed endothelial cells resulting in properdin serum levels of 4-25 μg/ml. Multiple inflammatory agonists stimulate the release of properdin from stimulated leukocytes into the cellular microenvironment. Concentrated, focused increases in properdin levels may lead to stabilization and initiation of alternative pathway convertases, thus greatly amplifying the complement response to a local stimulus. This review highlights current knowledge related to these properties and discusses the implications of properdin production in a pro-inflammatory microenvironment. [ABSTRACT FROM AUTHOR]
- Published
- 2012
- Full Text
- View/download PDF
21. Allotyping human complement factor B in Asian Indian type 1 diabetic patients.
- Author
-
Kumar, N., Kaur, G., Tandon, N., and Mehra, N. K.
- Subjects
- *
DIABETES , *MAJOR histocompatibility complex , *IMMUNE response , *GEL electrophoresis , *HLA histocompatibility antigens - Abstract
Human complement factor B (BF) is an essential component of the alternate complement pathway and therefore important in innate immune and autoimmune responses. The BF gene is located in the central region of major histocompatibility complex (MHC) and is known to encode more than 30 protein variants that can be resolved by isoelectric focusing and gel electrophoresis. There are three BF alleles – BF*S, BF*FB and BF*FA – that differ in codon 7 at nucleotide positions 94 and 95. These alleles have CGG, TGG or CAG triplets at their codon 7, respectively, that code for Arg, Trp or Gln residues. We have developed a novel polymerase chain reaction using sequence-specific primers-based allotyping assay that can identify nucleotide substitutions in codon 7 in all the three BF alleles. The assay was validated by sequencing and amplified fragment length polymorphism. Using this SSP assay, we report the BF alleles located on the multiple human leukocyte antigen (HLA)-DR3 haplotypes that are unique in the Indian population and are associated with autoimmunity. The common type 1 diabetes (T1D)-favoring Caucasian haplotype HLA-A1-B8-DR3 (ancestral haplotype AH8.1) carries BF*S. However, in the North Indian T1D patients, the most common haplotype is HLA-A26-B8-DR3 (AH8.2) and this carried BF*FB. Because of its association with AH8.2, the BF*FB was overrepresented in the patients (51.03%) compared with healthy controls (32.7%, OR = 2.148, 95% CI = 1.34–3.44, P = 0.002). Similar studies on allotyping BF alleles in different haplotypes in various populations could have important implications in understanding mechanisms of MHC haplotypic diversifications and disease associations and designing future therapeutic approaches. [ABSTRACT FROM AUTHOR]
- Published
- 2008
- Full Text
- View/download PDF
22. The alternative complement pathway activity may depend on plasma malondialdehyde level in systemic lupus erythematosus patients: Preliminary results
- Author
-
D. Batouche, Amina Boumediene, Fatma Haiba, and Kheir Eddine Kerboua
- Subjects
0301 basic medicine ,medicine.medical_specialty ,Oxygen radical absorbance capacity ,Inflammation ,medicine.disease_cause ,Pathogenesis ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Systemic lupus erythematosus ,Rheumatology ,Internal medicine ,Malondialdehyde ,medicine ,skin and connective tissue diseases ,Properdin factor b ,030203 arthritis & rheumatology ,Lipid peroxide ,business.industry ,030104 developmental biology ,Endocrinology ,chemistry ,Oxidative stress ,Alternative complement pathway ,medicine.symptom ,AH50 ,business - Abstract
Background Malondialdehyde (MDA) is a marker of oxidative stress (OS) and one of the major alternative complement pathway (ACP) activators associated with systemic lupus erythematosus (SLE) activity. ACP is the principal mediator of SLE inflammation and progression. Aim of the work To investigate the association between the ACP functional activity and plasma MDA in SLE patients. Patients and methods Sixteen consecutive SLE patients were analyzed for their complement profile and oxidative stress measurement. 60 healthy subjects were included as a control group. The Complement components C3, C4 and properdin-factor B (PfB) were assessed, ACP activity was assayed according to alternative hemolytic 50 (AH 50 ). Plasma total lipid peroxide quantification was performed by assessing the plasma MDA. Total antioxidant capacity was measured with oxygen radical absorbance capacity (ORAC). OS ratio was calculated by dividing the total antioxidant capacity by MDA. Results Sixteen patients (13 females and 3 males) with a mean age of 27.86 ± 6.26 years and a disease duration 69.65 ± 54.65 months were included. There was a significant increase of MDA in the patients (MFI = 613 ± 56.21) compared to the control (MFI = 460 ± 37.85) ( p = 0.003). C3 was significantly consumed and MDA increased in the low AH50 compared to the normal AH50 patients ( p = 0.02 and p = 0.01 respectively). AH50 significantly negatively correlated with the C3 ( p = 0.02) and MDA ( p = 0.048). There was lack of any association between ORAC and ACP. Properdin factor B significantly negatively correlated with C3 ( p = 0.007). Conclusions These initial results encourage future in-depth studies on the association of OS–ACP in SLE pathogenesis.
- Published
- 2016
- Full Text
- View/download PDF
23. Genetic Polymorphism of C3 and Bf in IgA Nephropathy.
- Author
-
Rambausek, M., van den Wall Bake, A. W. L., Schumacher-Ach, R., Spitzenberg, R., Rother, U., van Es, L. A., and Ritz, E.
- Abstract
C3 and Bf alleles were examined in the general population, in 67 patients with biopsy-confirmed mesangial IgA nephropathy and 81 patients with other types of glomerulonephritis, from the Heidelberg and Leiden renal programmes respectively.In both populations, a significant excess of homozygous phenotype C3FF (3.4% in controls; 10.4% in IgA nephropathy) and a deficit of C3FS heterozygous phenotype (35.8% in controls; 19.4% in IgA nephropathy) were observed in patients with IgA nephropathy, but not in other types of glomerulonephritis. No difference of C3 gene frequencies was found. C3FF was associated with an adverse clinical outcome (a higher prevalence of renal failure and hypertension).A significant excess of Bf-F gene frequency was noted (0.20 in controls; 0.33 in IgA nephropathy). In addition, an excess of phenotype BfFF was found (none in controls; 10.4% in IgA nephropathy). BfFF homozygotes also carried a higher risk of an adverse outcome (renal failure and hypertension).The data suggest a role for genetically coded (presumably) immunological factors in the genesis and course of IgA nephropathy. [ABSTRACT FROM PUBLISHER]
- Published
- 1987
24. The Bf system in diabetes-gene interaction or linkage disequilibrium?
- Author
-
Wolf, E., Cudworth, A., Markwick, J., Gorsuch, A., Spencer, K., and Bodansky, H.
- Abstract
HLA and Bf genotypes have been determined in 75 Type 1 (insulin-dependent) diabetic probands ascertained from two family studies. The Bf associations were similar in those families with two affected children compared with those families with one affected child. The strong association of B18 with the rare allele BfF1 was confirmed but, with rare exception, the gene encoding for BfF1 only occurred on chromosomes coding for both B18 and CW5. These findings are similar to the strong allelic association between these specificities found more frequently in Southern Europe and Spain, but no HLA-A locus association was found in the present study. All HLA-B 8 positive subjects and 15 out of 16 BW62 positive probands possessed BfS. In contrast to other reports, we could not find any evidence to support the idea that BfF1 is associated with a younger age of onset of diabetes. It is concluded that there is no specific susceptibility gene for Type 1 diabetes linked to the Bf locus and that the Bf associations can be explained purely on the basis of the known allelic association, or linkage disequilibrium, within the HLA system. [ABSTRACT FROM AUTHOR]
- Published
- 1982
- Full Text
- View/download PDF
25. Properdin factor B (Bf) polymorphism in the population of veneto, Italy.
- Author
-
Crestani, C., Caenazzo, L., Cortivo, P., Scorretti, C., and Caenazzo, C.
- Abstract
Copyright of Zeitschrift für Rechtsmedizin is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 1988
- Full Text
- View/download PDF
26. Properdin factor B-polymorphism.
- Author
-
Weidinger, S., Schwarzfischer, F., and Cleve, H.
- Abstract
Copyright of Zeitschrift für Rechtsmedizin is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 1979
- Full Text
- View/download PDF
27. Properdin factor B-polymorphism in the population of Hessen, Germany.
- Author
-
Kühnl, P. and Spielmann, W.
- Abstract
Copyright of Zeitschrift für Rechtsmedizin is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 1978
- Full Text
- View/download PDF
28. Complement genomics and antibody-mediated rejection in heart recipients.
- Author
-
Carbone, Javier
- Subjects
- *
GENOMICS , *IMMUNOGLOBULINS , *HEART transplantation , *SINGLE nucleotide polymorphisms , *PROPERDIN factor B - Abstract
In this issue of the Journal of Heart and Lung Transplantation , Marrón-Liñares et al report the results of an interesting study in which they evaluated 51 genes associated with the complement pathway in a small number of heart recipients to explore their relationship with antibody-mediated rejection (AMR). Next-generation sequencing was used in 46 heart transplant recipients (23 with AMR and 23 without AMR). The authors identified a significant association of 2 single-nucleotide polymorphisms with the absence or presence of AMR, respectively, p.Gly54Asp-MBL2 in the mannose-binding lectin (MBL) 2 gene and p.Asn428(p=)-CFP in the alternative complement factor properdin (CFP) gene. This article is a new contribution to the heart transplant literature. It suggests that complement single-nucleotide polymorphisms may influence circulating levels of selected proteins of both the lectin pathway and alternative complement pathways, thus potentially determining which patients will develop AMR. [ABSTRACT FROM AUTHOR]
- Published
- 2018
- Full Text
- View/download PDF
29. Genetic polymorphism of properdin factor B (BF) in domestic rabbit.
- Author
-
Branco, M., Lopes, G., and Ferrand, N.
- Subjects
- *
GENETIC polymorphisms , *ISOELECTRIC focusing , *ALLELES , *RABBITS , *GENETICS - Abstract
Genetic polymorphism of plasma properdin factor B (BF) was detected in domestic rabbit, Oryctolagus cuniculus , by means of isoelectric focusing and immunoblotting. The analysis of 298 individuals, corresponding to one French and two Portuguese populations, revealed the existence of six alleles, of which BF*A, B and C were common alleles, and D, F and G were rare ones. [ABSTRACT FROM AUTHOR]
- Published
- 1998
- Full Text
- View/download PDF
30. Properdin factor B in black Type 1 (insulin-dependent) diabetic patients.
- Author
-
Budowle, B., Reitnauer, P., Barger, B., Go, R., Roseman, J., and Acton, R.
- Abstract
The properdin factor B(Bf) variant F1 was found in 20% of 70 black patients with Type 1 (insulindependent) diabetes compared with 7.3% of 165 control subjects, yielding a significant relative risk of 3.1. In addition, the BfF allele was negatively associated with Type 1 diabetes among blacks, resulting in a relative risk of 0.4. The data suggest that Type 1 diabetes in black Americans may be partially due to admixture of Caucasoid genes. [ABSTRACT FROM AUTHOR]
- Published
- 1982
- Full Text
- View/download PDF
31. Association between HLA-A9 and rapidly progressive periodontitis
- Author
-
P. T. Klouda, Stephen Porter, Roger G. Smith, Crispian Scully, Benjamin A. Bradley, Roger Davies, and Susan A. Corbin
- Subjects
Periodontitis ,HLA-A Antigens ,business.industry ,Immunology ,General Medicine ,Immunogenetics ,Human leukocyte antigen ,medicine.disease ,Biochemistry ,Severe periodontitis ,White People ,Rapidly progressive periodontitis ,Gene Frequency ,Antigen ,HLA Antigens ,Genetics ,Humans ,Immunology and Allergy ,Medicine ,business ,Allele frequency ,Properdin factor b ,Complement Factor B - Abstract
HLA-A, B, C, DR antigen frequencies and Properdin factor B (Bf) allotypes were studied in a group of 44 patients with rapidly progressive periodontitis. HLA-A9 (A24) was the only antigen with a frequency statistically significantly different from the control population. An increased frequency of HLA-A9 was previously reported in periodontal diseases. Our results in a well characterised group of patients adds to the evidence that HLA-A9 plays a role in the susceptibility to severe periodontitis.
- Published
- 2008
32. Deficiency of C4 from brain death mice protects against renal injury.
- Author
-
Poppelaars, F., Jager, N.M., van Werkhoven, M.B., Damman, J., Hillebrand, J.L., Daha, M.R., Leuvenink, H.G.D., and Seelen, M.A.
- Subjects
- *
BRAIN death , *COMPLEMENT activation , *KIDNEY transplantation , *HEMODYNAMICS , *PROPERDIN factor B , *THERAPEUTICS - Published
- 2017
- Full Text
- View/download PDF
33. Crystallization and X‐ray analysis of monodisperse human properdin.
- Author
-
Pedersen, Dennis Vestergaard, Revel, Margot, Gadeberg, Trine Amalie Fogh, and Andersen, Gregers Rom
- Subjects
- *
PROPERDIN factor B , *CRYSTALLIZATION , *PROPROTEIN convertases - Abstract
The 54 kDa protein properdin, also known as factor P (FP), plays a major role in the complement system through the stabilization of the alternative pathway convertases. FP circulates in the blood as cyclic dimers, trimers and tetramers, and this heterogeneity challenges detailed structural insight into the mechanism of convertase stabilization by FP. Here, the generation of an intact FP monomer and a variant monomer with the third thrombospondin repeat liberated is described. Both FP monomers were excised from recombinant full‐length FP containing internal cleavage sites for TEV protease. These FP monomers could be crystallized, and complete data sets extending to 2.8 Å resolution for the intact FP monomer and to 3.5 Å resolution for the truncated variant were collected. The principle of specific monomer excision and domain removal by the insertion of a protease cleavage site may be broadly applicable to structural studies of oligomeric, flexible and modular proteins. A heterogeneous distribution of oligomers complicated the structure determination of the complement regulator properdin. Using TEV protease excision from recombinant oligomers, two types of properdin monomers could be crystallized. [ABSTRACT FROM AUTHOR]
- Published
- 2019
- Full Text
- View/download PDF
34. Properdin Factor B Alleles in Patients with Idiopathic Membranous Nephropathy
- Author
-
Netar P. Mallick, Philip A. Dyer, Rodney Harris, and P. T. Klouda
- Subjects
Enzyme Precursors ,business.industry ,Immunology ,General Medicine ,Biochemistry ,Idiopathic Membranous Nephropathy ,Gene Frequency ,HLA Antigens ,Genetics ,Humans ,Immunology and Allergy ,Medicine ,Kidney Diseases ,In patient ,Allele ,business ,Properdin factor b ,Alleles ,Complement Factor B - Published
- 2008
35. HLA-DR antigens and properdin factor B allotypes in responders and non-responders to the Rhesus-D antigen
- Author
-
J. Street, P. A. Dyer, C. Sargeant, and C. Darke
- Subjects
Male ,Complement Activating Enzymes ,Immunology ,Biochemistry ,Antigen ,Pregnancy ,Genetics ,Humans ,Immunology and Allergy ,Medicine ,Immunoglobulin Allotypes ,Properdin factor b ,HLA-DR Antigen ,Rh-Hr Blood-Group System ,business.industry ,Incidence (epidemiology) ,Histocompatibility Antigens Class II ,HLA-DR Antigens ,General Medicine ,medicine.disease ,Allotype ,Rhesus d ,Non responders ,Complement Factor D ,Female ,business - Abstract
HLA-DRw6 incidence was high in male responders to the Rhesus-D antigen (50%) compared to male non-responders (15.4%), P less than 0.02, P corrected N/S, pregnancy immunized females (23.1%) and a random population (26%). No Bf allotype frequency differences were found among these four groups.
- Published
- 2008
36. Polymorphism of properdin factor B (Bf) in some Italian populations
- Author
-
Fiorenzo Facchini, G. Gruppioni, P. Brasili Gualandi, and Donata Luiselli
- Subjects
Immunofixation ,S allele ,Veterinary medicine ,education.field_of_study ,biology ,Population ,General Medicine ,Anthropology ,Coomassie blue ,biology.protein ,Ethnology ,Animal Science and Zoology ,Cellulose acetate electrophoresis ,High incidence ,education ,Allele frequency ,Properdin factor b ,Ecology, Evolution, Behavior and Systematics - Abstract
In order to contribute to the definition of a map of the distribution of Bf polymorphism in Italy, we typed numerous population samples (for a total of 2033 individuals) from several regions of Italy: provinces of Bologna, Potenza and Matera, middle Sangro valley (Chieti) and northern, central and southern Sardinia. A new method was used which consisted of cellulose acetate electrophoresis followed by immunofixation and staining with Coomassie blue. The results obtained highlight marked differences in the distribution of Bf phenotypes and allele frequencies in Italy. To be noted in particular are the relatively high incidence of the Bf*S allele and the low incidence of Bf*F in Potenza and the significantly different pattern of the Sardinian samples compared to those from mainland Italy. This may be due to the low incidence in Sardinia of the Bf*S allele and above all to the exceptionally high frequency of the Bf*F1 variant.
- Published
- 1993
37. Genetic polymorphism of properdin factor B (BF) in domestic rabbit
- Author
-
M. Branco, G. Lopes, and Nuno Ferrand
- Subjects
Genetics ,Polymorphism, Genetic ,Portugal ,Isoelectric focusing ,Goats ,Blotting, Western ,Blood Proteins ,General Medicine ,Biology ,Phenotype ,Polymorphism (computer science) ,Animals, Domestic ,Animals ,Properdin ,Cattle ,Animal Science and Zoology ,France ,Rabbits ,Isoelectric Focusing ,Allele ,Gene ,Properdin factor b ,Alleles ,Complement Factor B - Abstract
Genetic polymorphism of plasma properdin factor B (BF) was detected in domestic rabbit, Oryctolagus cuniculus, by means of isoelectric focusing and immunoblotting. The analysis of 298 individuals, corresponding to one French and two Portuguese populations, revealed the existence of six alleles, of which BF*A, B and C were common alleles, and D, F and G were rare ones.
- Published
- 1998
38. Complement factor H-related (CFHR) proteins 2 and 5 as well as iC3b and properdin are deposited in different glomerular diseases.
- Author
-
Person, Fermin, Niebuhr, Norbert, Langbehn, Ulrike, Karaulashvili, Tamar, Rtskhiladze, Irakli, Thurman, Joshua M., Skerka, Christine, Rudnick, Ramona, Zipfel, Peter, and Wiech, Thorsten
- Subjects
- *
COMPLEMENT factor H , *KIDNEY disease treatments , *IMMUNOSTAINING , *PROPERDIN factor B , *RENAL biopsy - Published
- 2017
- Full Text
- View/download PDF
39. Structural studies of convertases in the complement system. The properdin-stabilised proconvertase, and the C5 convertase.
- Author
-
Gadeberg, Trine A.F., Pedersen, Dennis Vestergaard, and Andersen, Gregers Rom
- Subjects
- *
PROPROTEIN convertases , *PROPERDIN factor B , *COMPLEMENT (Immunology) , *FREEZE-drying , *COBRA venom factor - Published
- 2017
- Full Text
- View/download PDF
40. Properdin factor B allotype F and HLA-Dwl as protective markers in multiple sclerosis
- Author
-
M. Reunanen, J. Ilonen, A. Lagerstedt, and S. Koskimies
- Subjects
Neurology ,Multiple sclerosis ,Immunology ,medicine ,Neurology (clinical) ,General Medicine ,Human leukocyte antigen ,Biology ,medicine.disease ,Properdin factor b ,Allotype - Published
- 2009
41. The properdin factor B variant(BF S045)first detected in the Japanese population
- Author
-
Masakazu Oya, Noni Komatsu, Akira Kido, and Y. Kimura
- Subjects
Genetics ,Polymorphism (computer science) ,General Medicine ,Japanese population ,Biology ,Properdin factor b ,Complement (complexity) - Published
- 1990
42. Genetic polymorphisms in the fourth component of complement (C4) and in properdin factor B (BF) in Japanese patients with palmoplantar pustulosis
- Author
-
Yasumasa Ishibashi, Katsushi Tokunaga, Hidemi Nakagawa, Hideshi Torii, and Takeo Juji
- Subjects
Adult ,Male ,Palmoplantar pustulosis ,Polymorphism, Genetic ,Complement C4 ,Dermatology ,General Medicine ,Biology ,Middle Aged ,Complement (complexity) ,Pathogenesis ,Japan ,Immunology ,Humans ,Psoriasis ,Female ,Properdin factor b ,Aged ,Complement Factor B - Abstract
Genetic polymorphisms in the fourth component of complement (C4) and in properdin factor B (BF) were investigated in 49 and 32 Japanese patients with palmoplantar pustulosis (PPP), respectively. C4B2 was significantly increased in frequency, whereas no significant deviations were detected in BF compared with the controls. These results may indicate that complement polymorphisms are involved in the pathogenesis of PPP.
- Published
- 1992
43. Serum levels of ceruloplasmin, properdin factor B and copper in lymphoma patients
- Author
-
A Casamassima, Vito Lorusso, L Addabbo, and L Caporusso
- Subjects
Adult ,Male ,Cancer Research ,Pathology ,medicine.medical_specialty ,Adolescent ,Lymphoma ,Clinical Biochemistry ,chemistry.chemical_element ,Pathology and Forensic Medicine ,immune system diseases ,hemic and lymphatic diseases ,medicine ,Biomarkers, Tumor ,Humans ,Properdin factor b ,Aged ,biology ,business.industry ,Lymphoma, Non-Hodgkin ,Ceruloplasmin ,Middle Aged ,medicine.disease ,Copper ,Hodgkin Disease ,carbohydrates (lipids) ,Oncology ,chemistry ,biology.protein ,lipids (amino acids, peptides, and proteins) ,Female ,business ,Nephelometry ,Complement Factor B - Abstract
The serum levels of Ceruloplasmin (CER), Properdin Factor B (PFB) and Copper (CU) were evaluated in a series of 40 patients with Hodgkin's Disease and 46 patients with non-Hodgkin's lymphoma. Concentrations of CER and PFB were determined by rate nephelometry and CU concentrations by the bathocuproine colorimetric method. The results obtained demonstrated that CER, together with the well documented CU, can be used for monitoring Hodgkin's Disease.
- Published
- 1991
44. C3, BF and C4 polymorphisms in Tunisians
- Author
-
Kh. Ayed and Yousr Gorgi
- Subjects
Genetics ,Polymorphism, Genetic ,Tunisia ,Polymorphism (computer science) ,Tunisian population ,Humans ,Complement C4 ,Complement C3 ,Biology ,Properdin factor b ,Genetics (clinical) ,Complement Factor B - Abstract
The C3, BF, C4A and C4B polymorphisms were studied in a Tunisian population sample. The allelic frequencies for C3 were S = 0.844 and F = 0.148, and for BF, S = 0.535, F = 0.331, SO7 = 0.075 and F1 = 0.041. The most frequent C4 alleles were A3 and B1 followed in a decreasing order by A2, B2 and the A and B null alleles. The results indicate that the Tunisian population is intermediate between the Caucasian and Arab populations with some trace admixture of African Blacks.
- Published
- 1990
45. Infectious disease: Complementing antibacterial strategies.
- Author
-
Harrison, Charlotte
- Subjects
- *
PROPERDIN factor B , *STREPTOCOCCUS pneumoniae , *MOUSE diseases , *PREVENTION - Abstract
The article presents research showing the significance of recombinant properdin in protecting mice against Neisseria meningitides and Streptococcus pneumoniae infection.
- Published
- 2014
- Full Text
- View/download PDF
46. Properdin Factor B (Bf) Polymorphism in Haryana, India
- Author
-
S.M.S. Chahal and K.P.S. Kushwaha
- Subjects
Genetics ,Polymorphism (materials science) ,General Medicine ,Biology ,Properdin factor b - Published
- 1991
47. The BF locus and HLA: Rare alleles coding for functionally active and inactive factor-B products
- Author
-
Peggy Miniter, Soo Young Yang, Geoffrey J. O'Neill, Christoph Nerl, Marilyn S. Pollack, and B. Dupont
- Subjects
Genetics ,Enzyme Precursors ,Genotype ,Immunology ,Haplotype ,C4A ,Chromosome Mapping ,Locus (genetics) ,General Medicine ,Human leukocyte antigen ,Biology ,Complement factor B ,Molecular biology ,HLA Antigens ,Protein Biosynthesis ,Humans ,Immunology and Allergy ,Allele ,Immunoelectrophoresis ,Properdin factor b ,Alleles ,Complement Factor B - Abstract
The genetic polymorphism of properdin factor B (BF) was studied in different populations. The rarer alleles, BF*F1 and BF*S1, occurred in Caucasians, were less frequent in North American blacks, and were not demonstrated in any of three Oriental populations studied. Two further alleles of BF, termed BF*FM and BF*SM, were found to exist in these populations. The BF*FM allele, which was found only in Caucasians, codes for a functionally inactive factor-B product, whereas the BF*SM allele (found in a single Chinese individual), like other alleles of BF, codes for a functionally active product. HLA haplotype analyses in individuals carrying the rarer alleles of BF revealed not only a strong association between BF*F1 and HLA-B18 and BF*S1 and HLA-Bw50 but an even stronger association between these BF alleles and alleles of the two C4 loci. BF*F1 occurred most frequently on a C4A*3,B*Q0 haplotype, whereas the BF*S1 allele was usually found on a C4A*2,B*1/B*Q0 haplotype. HLA haplotypes carrying the BF*FM and BF*SM alleles all carried the more common C*4A3,B*1 haplotype.
- Published
- 1982
48. Polymorphism of properdin factor B in Japanese. Description of a rare variant and data of association with HLA and C2
- Author
-
Keiichi Omoto, C. Araki, Takeo Juji, and Katsushi Tokunaga
- Subjects
Adult ,Human leukocyte antigen ,Immunoelectrophoresis ,Biology ,Gene Frequency ,Japan ,HLA Antigens ,Genetic variation ,Genetics ,medicine ,Humans ,Allele ,Properdin factor b ,Allele frequency ,Alleles ,Genetics (clinical) ,Enzyme Precursors ,Polymorphism, Genetic ,medicine.diagnostic_test ,Genetic Variation ,Complement C2 ,Immunofixation electrophoresis ,Phenotype ,Complement Factor B - Abstract
Polymorphism of the properdin factor B (BF) was investigated using an agarose gel immunofixation electrophoresis in 487 unrelated healthy adult Japanese who were already typed for HLA-A, -B, -C and C2. Besides the previously reported phenotypes in Japanese (S, FS, and F), a rare heterozygous phenotype (tentatively maned FTS) was observed once. The estimated allele frequencies for BS*S, BF*F, and FB*FT (F Tokyo) were 0.801, 0.198, and 0,001 respectively. The relative electrophoretic mobility of the variant band of type FTS was measured by Dr. G Mauff to be F 0.75. The conversion fragment Bb of the type showed a double-banded pattern. BF hemolytic activity of the FTS individual was at the same level as other phenotypes. Statistical tests for the phenotypic data of BF with HLA-A, -B, -C, and C2 indicated the presence of the following significant associations in Japanese: Aw33-BF*F, A11-BF*S, Aw24-BF*S, B15-BF*F, B17-BF*F, Bw44-BF*F, B7-BF*S, Bw52;-BF*S, BW54-BF*S, BW59-BF*S, Cw3-BF*F, C2*AT-BF*F, and C2*A'-BF*F.
- Published
- 1982
49. Population genetic data on properdin factor B (Bf) in Northern Germany
- Author
-
Klaus Püschel, B. Brinkmann, R. Söder, and M. Schmidt
- Subjects
Adult ,Male ,Genetics ,Enzyme Precursors ,education.field_of_study ,Gene model ,Population ,Germany, West ,Genetic data ,Biology ,Phenotype ,Gene Frequency ,Anthropology ,Humans ,Female ,Anatomy ,Child ,education ,Gene ,Properdin factor b ,Alleles ,Complement Factor B ,Genes, Dominant - Abstract
Properdin factor B phenotypes were determined in 1,112 unrelated individuals and in 151 mother/child combinations from Northern Germany. Gene frequencies were : F = 0.1960, S= 0.7905, F1 = 0.0072, S1 = 0.0063. The data of the mother/child combinations are in full accordance with the postulated gene model.
- Published
- 1979
50. Properdin Factor B (Bf) Polymorphism in Norway
- Author
-
Per Teisberg and Bjørnar Olaisen
- Subjects
Genetics ,education.field_of_study ,Polymorphism, Genetic ,Properdin ,Norway ,Population ,Norwegian ,Hematology ,General Medicine ,Biology ,language.human_language ,Phenotype ,Polymorphism (computer science) ,language ,Humans ,Allele ,education ,Allele frequency ,Properdin factor b ,Alleles - Abstract
Bf phenotype distribution and Bf allele frequencies in samples of the Norwegian population and the Lappish minority of Norway are presented. Bfs in Norwegians was found to be 0.817, while it was 0.888 in Lapps. The difference is statistically significant. Possible explanations of this difference are discussed. It is noted that Bf allele frequencies of Norwegians are similar to those of Germans, while differing from frequencies found in US whites.
- Published
- 1977
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