LIVIVO - Search results -

Search results

Result 1 - 10 of total 22

Search options

Article ; Online: Detection of Autoantibodies to Complement Components by Surface Plasmon Resonance-Based Technology.

Noe, Remi / Chauvet, Sophie / Togarsimalemath, Shambhuprasad K / Marinozzi, Maria Chiara / Radanova, Maria / Vasilev, Vasil V / Fremeaux-Bacchi, Veronique / Dragon-Durey, Marie-Agnes / Roumenina, Lubka T

Methods in molecular biology (Clifton, N.J.)

2018 Volume 1901, Page(s) 271–280

Abstract: The innate immune complement system is a powerful defense cascade against pathogens, but can induce host tissue damage when overactivated. In pathological conditions, mainly but not restricted to renal diseases, such as lupus nephritis, atypical ... ...

Abstract	The innate immune complement system is a powerful defense cascade against pathogens, but can induce host tissue damage when overactivated. In pathological conditions, mainly but not restricted to renal diseases, such as lupus nephritis, atypical hemolytic uremic syndrome, and C3 glomerulopathies, complement is overactivated or dysregulated by autoantibodies directed against its components and regulators. Among the key autoantibody targets are the initiator of the classical complement pathway C1q, the alternative pathway regulator Factor H, the components of the alternative pathway C3 convertase complex C3 and Factor B and the convertase complex itself. This methodological article describes our experience with a method for detection of anti-complement autoantibodies in real time using surface plasmon resonance-based technology. It allows label-free evaluation of the binding efficacy and stability of the formed antigen-antibody complexes.
MeSH term(s)	Antigens/metabolism ; Autoantibodies/analysis ; Complement System Proteins/immunology ; Dialysis ; Humans ; Immunoglobulin G/isolation & purification ; Surface Plasmon Resonance/methods
Chemical Substances	Antigens ; Autoantibodies ; Immunoglobulin G ; Complement System Proteins (9007-36-7)
Language	English
Publishing date	2018-12-11
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1940-6029
ISSN (online)	1940-6029
DOI	10.1007/978-1-4939-8949-2_24
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

Order via subito

This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

Inter-library loan at ZB MED

Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

Details ▾
- Full text online
- Order with fees

Article ; Online: Autoantibodies against complement components and functional consequences.

Dragon-Durey, Marie-Agnès / Blanc, Caroline / Marinozzi, Maria Chiara / van Schaarenburg, Rosanne A / Trouw, Leendert A

Molecular immunology

2013 Volume 56, Issue 3, Page(s) 213–221

Abstract: The complement system represents a major component of our innate immune defense. Although the physiological contribution of the complement system is beneficial, it can cause tissue damage when inappropriately activated or when it is a target of an ... ...

Abstract	The complement system represents a major component of our innate immune defense. Although the physiological contribution of the complement system is beneficial, it can cause tissue damage when inappropriately activated or when it is a target of an autoantibody response. Autoantibodies directed against a variety of individual complement components, convertases, regulators and receptors have been described. For several autoantibodies the functional consequences are well documented and clear associations exist with clinical presentation, whereas for other autoantibodies targeting complement components this relation is currently insufficiently clear. Several anti-complement autoantibodies can also be detected in healthy controls, indicating that a second hit is required for such autoantibodies to induce or participate in pathology or alternatively that these antibodies are part of the natural antibody repertoire. In the present review, we describe autoantibodies against complement components and their functional consequences and discuss about their clinical relevance.
MeSH term(s)	Animals ; Autoantibodies/immunology ; Complement C3-C5 Convertases/immunology ; Complement Factor H/immunology ; Complement Pathway, Alternative/immunology ; Complement Pathway, Classical/immunology ; Complement Pathway, Mannose-Binding Lectin/immunology ; Complement System Proteins/immunology ; Humans ; Receptors, Complement/immunology
Chemical Substances	Autoantibodies ; Receptors, Complement ; Complement Factor H (80295-65-4) ; Complement System Proteins (9007-36-7) ; Complement C3-C5 Convertases (EC 3.4.21.-)
Language	English
Publishing date	2013-12-15
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	424427-8
ISSN	1872-9142 ; 0161-5890
ISSN (online)	1872-9142
ISSN	0161-5890
DOI	10.1016/j.molimm.2013.05.009
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 166: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Innovative approach to safely induce controlled lipolysis by superparamagnetic iron oxide nanoparticles-mediated hyperthermic treatment.

Marinozzi, Maria Rosaria / Pandolfi, Laura / Malatesta, Manuela / Colombo, Miriam / Collico, Veronica / Lievens, Patricia Marie-Jeanne / Tambalo, Stefano / Lasconi, Chiara / Vurro, Federica / Boschi, Federico / Mannucci, Silvia / Sbarbati, Andrea / Prosperi, Davide / Calderan, Laura

The international journal of biochemistry & cell biology

2017 Volume 93, Page(s) 62–73

Abstract: During last years, evidence has been provided on the involvement of overweight and obesity in the pathogenesis and aggravation of several life-threatening diseases. Here, we demonstrate that, under appropriate administration conditions, polyhedral iron ... ...

Abstract	During last years, evidence has been provided on the involvement of overweight and obesity in the pathogenesis and aggravation of several life-threatening diseases. Here, we demonstrate that, under appropriate administration conditions, polyhedral iron oxide nanoparticles are efficiently and safely taken up by 3T3 cell line-derived adipocytes (3T3 adipocytes) in vitro. Since these nanoparticles proved to effectively produce heat when subjected to alternating magnetic field, 3T3 adipocytes were submitted to superparamagnetic iron oxide nanoparticles-mediated hyperthermia treatment (SMHT), with the aim of modulating their lipid content. Notably, the treatment resulted in a significant delipidation persisting for at least 24h, and in the absence of cell death, damage or dedifferentiation. Interestingly, transcript expression of adipose triglyceride lipase (ATGL), a key gene involved in canonical lipolysis, was not modulated upon SMHT, suggesting the involvement of a novel/alternative mechanism in the effective lipolysis observed. By applying the same experimental conditions successfully used for 3T3 adipocytes, SMHT was able to induce delipidation also in primary cultures of human adipose-derived adult stem cells. The success of this pioneering approach in vitro opens promising perspectives for the application of SMHT in vivo as an innovative safe and physiologically mild strategy against obesity, potentially useful in association with balanced diet and healthy lifestyle.
Language	English
Publishing date	2017-10-27
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	1228429-4
ISSN	1878-5875 ; 1357-2725
ISSN (online)	1878-5875
ISSN	1357-2725
DOI	10.1016/j.biocel.2017.10.013
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 431: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Heterogeneous histologic and clinical evolution in 3 cases of dense deposit disease with long-term follow-up.

Figuères, Marie-Lucile / Frémeaux-Bacchi, Véronique / Rabant, Marion / Galmiche, Louise / Marinozzi, Maria Chiara / Grünfeld, Jean-Pierre / Noël, Laure-Hélène / Servais, Aude

Human pathology

2014 Volume 45, Issue 11, Page(s) 2326–2333

Abstract: Dense deposit disease is characterized by dense deposits in the glomerular and tubular basement membranes. We report 3 cases with long-term follow-up differing in histologic pattern and clinical evolution. Clinical and histologic data were collected ... ...

Abstract	Dense deposit disease is characterized by dense deposits in the glomerular and tubular basement membranes. We report 3 cases with long-term follow-up differing in histologic pattern and clinical evolution. Clinical and histologic data were collected between 1976 and 2012. Age at the first manifestations was 6, 11, and 23 years, respectively. They included proteinuria (patient 1) and nephrotic syndrome (patients 2 and 3); renal function was normal in all cases. Two patients (1 and 3) had low complement component 3 (C3) levels. All patients had C3 nephritic factor. Genetic analysis revealed a rare variant of the factor I gene (patient 1) and a heterozygous mutation in complement factor H-related 5 gene (patient 2). Patient 1 underwent 3 biopsies during her 38 years of follow-up. Thickening of the capillary walls of the glomerular and tubular basement membranes was observed, with mild mesangial proliferation and progressive C3 and complement membrane attack complex mesangial deposits. However, renal function remained normal. Patient 2 also underwent 3 biopsies (22 years of follow-up), revealing a gradual decrease in C3 deposition and mesangial cell proliferation. He presented mild renal insufficiency. Patient 3 underwent 2 biopsies, which displayed unusual bulky membranous deposits, confirmed by electron microscopy, with no mesangial cell proliferation and little C3 and complement membrane attack complex deposits. Kidney function remained normal. These 3 cases of dense deposit disease differed in histologic pattern evolution: accumulation of C3 deposits, decrease in C3 deposits and proliferation, and isolated dense deposits. The histologic factors involved in clinical progression remain to be identified.
MeSH term(s)	Adolescent ; Adult ; Child ; Disease Progression ; Female ; Follow-Up Studies ; Glomerulonephritis/pathology ; Glomerulonephritis, Membranoproliferative/pathology ; Humans ; Kidney Glomerulus/pathology ; Male ; Nephrotic Syndrome/pathology ; Renal Insufficiency/pathology ; Young Adult
Language	English
Publishing date	2014-11
Publishing country	United States
Document type	Journal Article
ZDB-ID	207657-3
ISSN	1532-8392 ; 0046-8177
ISSN (online)	1532-8392
ISSN	0046-8177
DOI	10.1016/j.humpath.2014.07.021
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 722: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Both Monoclonal and Polyclonal Immunoglobulin Contingents Mediate Complement Activation in Monoclonal Gammopathy Associated-C3 Glomerulopathy.

Chauvet, Sophie / Roumenina, Lubka T / Aucouturier, Pierre / Marinozzi, Maria-Chiara / Dragon-Durey, Marie-Agnès / Karras, Alexandre / Delmas, Yahsou / Le Quintrec, Moglie / Guerrot, Dominique / Jourde-Chiche, Noémie / Ribes, David / Ronco, Pierre / Bridoux, Frank / Fremeaux-Bacchi, Véronique

Frontiers in immunology

2018 Volume 9, Page(s) 2260

Abstract: C3 glomerulopathy (C3G) results from acquired or genetic abnormalities in the complement alternative pathway (AP). C3G with monoclonal immunoglobulin (MIg-C3G) was recently included in the spectrum of "monoclonal gammopathy of renal significance." ... ...

Abstract	C3 glomerulopathy (C3G) results from acquired or genetic abnormalities in the complement alternative pathway (AP). C3G with monoclonal immunoglobulin (MIg-C3G) was recently included in the spectrum of "monoclonal gammopathy of renal significance." However, mechanisms of complement dysregulation in MIg-C3G are not described and the pathogenic effect of the monoclonal immunoglobulin is not understood. The purpose of this study was to investigate the mechanisms of complement dysregulation in a cohort of 41 patients with MIg-C3G. Low C3 level and elevated sC5b-9, both biomarkers of C3 and C5 convertase activation, were present in 44 and 78% of patients, respectively. Rare pathogenic variants were identified in 2/28 (7%) tested patients suggesting that the disease is acquired in a large majority of patients. Anti-complement auto-antibodies were found in 20/41 (49%) patients, including anti-FH (17%), anti-CR1 (27%), anti-FI (5%) auto-antibodies, and C3 Nephritic Factor (7%) and were polyclonal in 77% of patients. Using cofactor assay, the regulation of the AP was altered in presence of purified IgG from 3/9 and 4/7 patients with anti-FH or anti-CR1 antibodies respectively. By using fluid and solid phase AP activation, we showed that total purified IgG of 22/34 (65%) MIg-C3G patients were able to enhance C3 convertase activity. In five documented cases, we showed that the C3 convertase enhancement was mostly due to the monoclonal immunoglobulin, thus paving the way for a new mechanism of complement dysregulation in C3G. All together the results highlight the contribution of both polyclonal and monoclonal Ig in MIg-C3G. They provide direct insights to treatment approaches and opened up a potential way to a personalized therapeutic strategy based on chemotherapy adapted to the B cell clone or immunosuppressive therapy.
MeSH term(s)	Adult ; Complement C3/immunology ; Complement C3/metabolism ; Complement C5/immunology ; Complement C5/metabolism ; Complement Pathway, Alternative/immunology ; Female ; Glomerulonephritis/blood ; Glomerulonephritis/immunology ; Glomerulonephritis/pathology ; Humans ; Immunoglobulin G/blood ; Immunoglobulin G/immunology ; Male ; Middle Aged ; Paraproteinemias/blood ; Paraproteinemias/immunology ; Paraproteinemias/pathology
Chemical Substances	Complement C3 ; Complement C5 ; Immunoglobulin G
Language	English
Publishing date	2018-10-02
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2018.02260
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article: Innovative approach to safely induce controlled lipolysis by superparamagnetic iron oxide nanoparticles-mediated hyperthermic treatment

Marinozzi, Maria Rosaria / Andrea Sbarbati / Chiara Lasconi / Davide Prosperi / Federica Vurro / Federico Boschi / Laura Calderan / Laura Pandolfi / Manuela Malatesta / Miriam Colombo / Patricia Marie-Jeanne Lievens / Silvia Mannucci / Stefano Tambalo / Veronica Collico

international journal of biochemistry & cell biology. 2017 Dec., v. 93

2017

Abstract	During last years, evidence has been provided on the involvement of overweight and obesity in the pathogenesis and aggravation of several life-threatening diseases. Here, we demonstrate that, under appropriate administration conditions, polyhedral iron oxide nanoparticles are efficiently and safely taken up by 3T3 cell line-derived adipocytes (3T3 adipocytes) in vitro. Since these nanoparticles proved to effectively produce heat when subjected to alternating magnetic field, 3T3 adipocytes were submitted to superparamagnetic iron oxide nanoparticles-mediated hyperthermia treatment (SMHT), with the aim of modulating their lipid content. Notably, the treatment resulted in a significant delipidation persisting for at least 24h, and in the absence of cell death, damage or dedifferentiation. Interestingly, transcript expression of adipose triglyceride lipase (ATGL), a key gene involved in canonical lipolysis, was not modulated upon SMHT, suggesting the involvement of a novel/alternative mechanism in the effective lipolysis observed. By applying the same experimental conditions successfully used for 3T3 adipocytes, SMHT was able to induce delipidation also in primary cultures of human adipose-derived adult stem cells. The success of this pioneering approach in vitro opens promising perspectives for the application of SMHT in vivo as an innovative safe and physiologically mild strategy against obesity, potentially useful in association with balanced diet and healthy lifestyle.
Keywords	adipocytes ; adult stem cells ; cell death ; diet ; fever ; genes ; heat ; humans ; iron oxides ; lifestyle ; lipid content ; lipolysis ; magnetic fields ; nanoparticles ; obesity ; pathogenesis ; triacylglycerol lipase
Language	English
Dates of publication	2017-12
Size	p. 62-73.
Publishing place	Elsevier Ltd
Document type	Article
ZDB-ID	1228429-4
ISSN	1878-5875 ; 1357-2725
ISSN (online)	1878-5875
ISSN	1357-2725
DOI	10.1016/j.biocel.2017.10.013
Database	NAL-Catalogue (AGRICOLA)

In stock of ZB MED Cologne/Königswinter

Zs.A 431: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article: Politiche professionali: studio qualitativo sulle percezioni e le opinioni dei professionisti infermieri/ostetriche e degli studenti della Laurea magistrale in Scienze infermieristiche e ostetriche.

Professioni infermieristiche

2019 Volume 71, Issue 4, Page(s) 221–231

Abstract: Introduction: Politics, the science and the art of administering public affairs, is a very important field. Dealing with professional policy means to dedicate ourselves to the good of the profession. Professional policies are an integral part of taking ... ...

Title translation	Professional policies: a qualitative study of perceptions and opinions of professionals nurses/midwives and students attending the Master of Science in Nursing.
Abstract	Introduction: Politics, the science and the art of administering public affairs, is a very important field. Dealing with professional policy means to dedicate ourselves to the good of the profession. Professional policies are an integral part of taking care; the professional family needs clear reference points: training, tutoring, promotion and institutional representation. Aim: Exploring perceptions, ideals, shared imagination and desires about the professional policies of nurses/midwives and of master's students in order to be able to have a representation of the reality and to start a critical reflection on the subject. Method: A qualitative study was conducted on a proactive sample of 22 nurses / midwives as qualified witnesses of the national situation. The data was collected through a questionnaire built ad hoc. Results: From the analysis of the answers given to the questionnaire, we identified 10 main themes. In the training sector two of the themes that were selected are the importance of the new knowledges and inadequate differentiation of the positions; Regarding the working sector two of the themes that came out are the lack of meritocracy and of new opportunities; meanwhile, in the research field one of the main theme identified is the gap between the theory and the practice. Conclusion: The present study finds out that participation in policy-making processes is occasional and of little intensity: just few nurses/midwives are actively involved in the promotion and development of projects in the health sector and of healthcare policies.
MeSH term(s)	Adult ; Female ; Health Policy ; Humans ; Male ; Middle Aged ; Nurse Midwives/organization & administration ; Nurse Midwives/statistics & numerical data ; Nurses/organization & administration ; Nurses/statistics & numerical data ; Organizational Policy ; Policy Making ; Politics ; Qualitative Research ; Students, Nursing/statistics & numerical data ; Surveys and Questionnaires ; Young Adult
Language	Italian
Publishing date	2019-04-13
Publishing country	Italy
Document type	Journal Article
ZDB-ID	1217777-5
ISSN	0033-0205
ISSN	0033-0205
DOI	10.7429/pi.2018.714221
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 4189: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 636: Show issues

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Anti-Factor B and Anti-C3b Autoantibodies in C3 Glomerulopathy and Ig-Associated Membranoproliferative GN.

Marinozzi, Maria Chiara / Roumenina, Lubka T / Chauvet, Sophie / Hertig, Alexandre / Bertrand, Dominique / Olagne, Jérome / Frimat, Marie / Ulinski, Tim / Deschênes, Georges / Burtey, Stephane / Delahousse, Michel / Moulin, Bruno / Legendre, Christophe / Frémeaux-Bacchi, Véronique / Le Quintrec, Moglie

Journal of the American Society of Nephrology : JASN

2017 Volume 28, Issue 5, Page(s) 1603–1613

Abstract: In C3 glomerulopathy (C3G), the alternative pathway of complement is frequently overactivated by autoantibodies that stabilize the C3 convertase C3bBb. Anti-C3b and anti-factor B (anti-FB) IgG have been reported in three patients with C3G. We screened a ... ...

Abstract	In C3 glomerulopathy (C3G), the alternative pathway of complement is frequently overactivated by autoantibodies that stabilize the C3 convertase C3bBb. Anti-C3b and anti-factor B (anti-FB) IgG have been reported in three patients with C3G. We screened a cohort of 141 patients with C3G and Ig-associated membranoproliferative GN (Ig-MPGN) for anti-FB and anti-C3b autoantibodies using ELISA. We identified seven patients with anti-FB IgG, three patients with anti-C3b IgG, and five patients with anti-FB and anti-C3b IgG. Of these 15 patients, ten were diagnosed with Ig-MPGN. Among those patients with available data, 92% had a nephrotic syndrome, 64% had AKI, and 67% had a documented infection. Patients negative for anti-C3b and anti-FB IgG had much lower rates of infection (17 [25%] patients with C3G and one [10%] patient with Ig-MPGN). After 48 months, four of 15 (26%) positive patients had developed ESRD or died. All 15 patients had high plasma Bb levels, six (40%) patients had low levels of C3, and nine (60%) patients had high levels of soluble C5b9.
MeSH term(s)	Adolescent ; Adult ; Autoantibodies/immunology ; Child ; Complement C3b/immunology ; Complement Factor B/immunology ; Female ; Glomerulonephritis, Membranoproliferative/immunology ; Humans ; Immunoglobulin G/immunology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult
Chemical Substances	Autoantibodies ; Immunoglobulin G ; Complement C3b (80295-43-8) ; Complement Factor B (EC 3.4.21.47)
Language	English
Publishing date	2017-05
Publishing country	United States
Document type	Journal Article
ZDB-ID	1085942-1
ISSN	1533-3450 ; 1046-6673
ISSN (online)	1533-3450
ISSN	1046-6673
DOI	10.1681/ASN.2016030343
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 3344: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: C5 nephritic factors drive the biological phenotype of C3 glomerulopathies.

Marinozzi, Maria-Chiara / Chauvet, Sophie / Le Quintrec, Moglie / Mignotet, Morgane / Petitprez, Florent / Legendre, Christophe / Cailliez, Mathilde / Deschenes, Georges / Fischbach, Michel / Karras, Alexandre / Nobili, Francois / Pietrement, Christine / Dragon-Durey, Marie-Agnes / Fakhouri, Fadi / Roumenina, Lubka T / Fremeaux-Bacchi, Veronique

Kidney international

2017 Volume 92, Issue 5, Page(s) 1232–1241

Abstract: C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis, are associated with genetic and acquired dysregulation of the C3 convertase alternative pathway of complement. The potential role of the activation of the C5 convertase ... ...

Abstract	C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis, are associated with genetic and acquired dysregulation of the C3 convertase alternative pathway of complement. The potential role of the activation of the C5 convertase has not been studied extensively. Here we analyzed IgG samples from patients with C3 Glomerulopathies to identify circulating autoantibodies that stabilize the C3 alternative pathway (C3 Nephritic Factors) as well as C5 convertases (C5 Nephritic Factors), thus preventing decay of these enzyme complexes. Rare variants in alternative pathway genes were found in 28 of 120 tested patients. C3 and C5 Nephritic Factors were found in 76 of 101 (75%) and 29 of 59 (49%) of the patients, respectively. Therefore, we compared the results of the assays for the C3 and C5 nephritic factors functional activity: 29% were positive for C3 Nephritic Factors alone, 39% were positive for both C3 and C5 Nephritic Factors, and 10% were positive for C5 Nephritic Factors alone. We found that the addition of properdin-enhanced stabilization of C3 convertase in the presence of IgG doubly positive for both Nephritic Factors, while it did not modify the stabilization mediated by IgG solely positive for C3 Nephritic Factors. Both C3 and C5 Nephritic Factors correlated with C3 consumption, while only C5 Nephritic Factors correlated with sC5b9 levels. C5 Nephritic Factors-positive patients were more likely to have C3 Glomerulonephritis than Dense Deposit Disease. Thus, dysregulation of the C5 convertase contributes to C3 Glomerulopathies inter-disease differences and may have direct therapeutic implications.
MeSH term(s)	Adolescent ; Adult ; Child ; Complement C3 Convertase, Alternative Pathway/immunology ; Complement C3 Nephritic Factor/analysis ; Complement C3 Nephritic Factor/genetics ; Complement C3 Nephritic Factor/immunology ; Complement C3-C5 Convertases/immunology ; Complement C3-C5 Convertases/metabolism ; Complement Membrane Attack Complex/analysis ; Complement Pathway, Alternative/immunology ; Female ; Follow-Up Studies ; Glomerulonephritis, Membranoproliferative/blood ; Glomerulonephritis, Membranoproliferative/genetics ; Glomerulonephritis, Membranoproliferative/immunology ; Glomerulonephritis, Membranoproliferative/mortality ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Phenotype ; Properdin/metabolism ; Serologic Tests ; Young Adult
Chemical Substances	Complement C3 Nephritic Factor ; Complement Membrane Attack Complex ; SC5b-9 protein complex ; Properdin (11016-39-0) ; Complement C3-C5 Convertases (EC 3.4.21.-) ; Complement C3 Convertase, Alternative Pathway (EC 3.4.21.47)
Language	English
Publishing date	2017-07-14
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2017.04.017
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 797: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H.

Chauvet, Sophie / Roumenina, Lubka T / Bruneau, Sarah / Marinozzi, Maria Chiara / Rybkine, Tania / Schramm, Elizabeth C / Java, Anuja / Atkinson, John P / Aldigier, Jean Claude / Bridoux, Frank / Touchard, Guy / Fremeaux-Bacchi, Veronique

Journal of the American Society of Nephrology : JASN

2015 Volume 27, Issue 6, Page(s) 1665–1677

Abstract: C3 glomerulopathy is a recently described form of CKD. C3GN is a subtype of C3 glomerulopathy characterized by predominant C3 deposits in the glomeruli and is commonly the result of acquired or genetic abnormalities in the alternative pathway (AP) of the ...

Abstract	C3 glomerulopathy is a recently described form of CKD. C3GN is a subtype of C3 glomerulopathy characterized by predominant C3 deposits in the glomeruli and is commonly the result of acquired or genetic abnormalities in the alternative pathway (AP) of the complement system. We identified and characterized the first mutation of the C3 gene (p. I734T) in two related individuals diagnosed with C3GN. Immunofluorescence and electron microscopy studies showed C3 deposits in the subendothelial space, associated with unusual deposits located near the complement receptor 1 (CR1)-expressing podocytes. In vitro, this C3 mutation exhibited decreased binding to CR1, resulting in less CR1-dependent cleavage of C3b by factor 1. Both patients had normal plasma C3 levels, and the mutant C3 interacted with factor B comparably to wild-type (WT) C3 to form a C3 convertase. Binding of mutant C3 to factor H was normal, but mutant C3 was less efficiently cleaved by factor I in the presence of factor H, leading to enhanced C3 fragment deposition on glomerular cells. In conclusion, our results reveal that a CR1 functional deficiency is a mechanism of intraglomerular AP dysregulation and could influence the localization of the glomerular C3 deposits.
MeSH term(s)	Adult ; Complement C3/genetics ; Complement Factor H/genetics ; Gene Expression Regulation ; Glomerulonephritis, Membranoproliferative/genetics ; Humans ; Male ; Middle Aged ; Pedigree ; Receptors, Complement 3b/genetics
Chemical Substances	CFH protein, human ; Complement C3 ; Receptors, Complement 3b ; Complement Factor H (80295-65-4)
Language	English
Publishing date	2015-10-15
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	1085942-1
ISSN	1533-3450 ; 1046-6673
ISSN (online)	1533-3450
ISSN	1046-6673
DOI	10.1681/ASN.2015040348
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 3344: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

To top

Full text online

More links

Kategorien

Order via subito

Inter-library loan at ZB MED

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito