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Article ; Online: XXIX International Complement Workshop Newcastle 2023.

Marchbank, Kevin J / Kavanagh, David / Harris, Claire L

2023 Volume 228, Issue 5, Page(s) 152729

Language	English
Publishing date	2023-08-10
Publishing country	Netherlands
Document type	Editorial
ZDB-ID	563292-4
ISSN	1878-3279 ; 0171-2985
ISSN (online)	1878-3279
ISSN	0171-2985
DOI	10.1016/j.imbio.2023.152729
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Article ; Online: Contribution of animal models to the mechanistic understanding of Alternative Pathway and Amplification Loop (AP/AL)-driven Complement-mediated Diseases.

Gibson, Beth G / Cox, Thomas E / Marchbank, Kevin J

Immunological reviews

2022 Volume 313, Issue 1, Page(s) 194–216

Abstract: This review aimed to capture the key findings that animal models have provided around the role of the alternative pathway and amplification loop (AP/AL) in disease. Animal models, particularly mouse models, have been incredibly useful to define the role ... ...

Abstract	This review aimed to capture the key findings that animal models have provided around the role of the alternative pathway and amplification loop (AP/AL) in disease. Animal models, particularly mouse models, have been incredibly useful to define the role of complement and the alternative pathway in health and disease; for instance, the use of cobra venom factor and depletion of C3 provided the initial insight that complement was essential to generate an appropriate adaptive immune response. The development of knockout mice have further underlined the importance of the AP/AL in disease, with the FH knockout mouse paving the way for the first anti-complement drugs. The impact from the development of FB, properdin, and C3 knockout mice closely follows this in terms of mechanistic understanding in disease. Indeed, our current understanding that complement plays a role in most conditions at one level or another is rooted in many of these in vivo studies. That C3, in particular, has roles beyond the obvious in innate and adaptive immunity, normal physiology, and cellular functions, with or without other recognized AP components, we would argue, only extends the reach of this arm of the complement system. Humanized mouse models also continue to play their part. Here, we argue that the animal models developed over the last few decades have truly helped define the role of the AP/AL in disease.
MeSH term(s)	Animals ; Mice ; Humans ; Complement Pathway, Alternative ; Properdin/metabolism ; Adaptive Immunity ; Mice, Knockout ; Disease Models, Animal
Chemical Substances	Properdin (11016-39-0)
Language	English
Publishing date	2022-10-06
Publishing country	England
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	391796-4
ISSN	1600-065X ; 0105-2896
ISSN (online)	1600-065X
ISSN	0105-2896
DOI	10.1111/imr.13141
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Article ; Online: Contribution of animal models to the mechanistic understanding of Alternative Pathway and Amplification Loop (AP/AL)‐driven Complement‐mediated Diseases

Gibson, Beth G. / Cox, Thomas E. / Marchbank, Kevin J.

Immunological Reviews. 2023 Jan., v. 313, no. 1, p. 194-216

2023 , Page(s) 194–216

Abstract	This review aimed to capture the key findings that animal models have provided around the role of the alternative pathway and amplification loop (AP/AL) in disease. Animal models, particularly mouse models, have been incredibly useful to define the role of complement and the alternative pathway in health and disease; for instance, the use of cobra venom factor and depletion of C3 provided the initial insight that complement was essential to generate an appropriate adaptive immune response. The development of knockout mice have further underlined the importance of the AP/AL in disease, with the FH knockout mouse paving the way for the first anti‐complement drugs. The impact from the development of FB, properdin, and C3 knockout mice closely follows this in terms of mechanistic understanding in disease. Indeed, our current understanding that complement plays a role in most conditions at one level or another is rooted in many of these in vivo studies. That C3, in particular, has roles beyond the obvious in innate and adaptive immunity, normal physiology, and cellular functions, with or without other recognized AP components, we would argue, only extends the reach of this arm of the complement system. Humanized mouse models also continue to play their part. Here, we argue that the animal models developed over the last few decades have truly helped define the role of the AP/AL in disease.
Keywords	adaptive immunity ; cobra venoms ; complement ; knockout mutants ; mice ; physiology
Language	English
Dates of publication	2023-01
Size	p. 194-216
Publishing place	John Wiley & Sons, Ltd
Document type	Article ; Online
Note	REVIEW
ZDB-ID	391796-4
ISSN	1600-065X ; 0105-2896
ISSN (online)	1600-065X
ISSN	0105-2896
DOI	10.1111/imr.13141
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Article ; Online: Editorial: Autoantibodies in Kidney Diseases.

Marchbank, Kevin J / Frazer-Abel, Ashley / Dragon-Durey, Marie-Agnes / Dixon, Bradley P

Frontiers in immunology

2020 Volume 11, Page(s) 591338

MeSH term(s)	Autoantibodies/immunology ; Autoimmune Diseases/etiology ; Autoimmune Diseases/metabolism ; Autoimmune Diseases/pathology ; Autoimmunity ; Disease Management ; Disease Susceptibility ; Humans ; Kidney Diseases/etiology ; Kidney Diseases/metabolism ; Kidney Diseases/pathology
Chemical Substances	Autoantibodies
Language	English
Publishing date	2020-09-16
Publishing country	Switzerland
Document type	Editorial ; Introductory 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.2020.591338
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Article: Targeting properdin in the treatment of atypical haemolytic uraemic syndrome: better than eculizumab?

Smith-Jackson, Kate / Marchbank, Kevin J

Annals of translational medicine

2017 Volume 6, Issue Suppl 1, Page(s) S62

Language	English
Publishing date	2017-11-21
Publishing country	China
Document type	Editorial ; Comment
ZDB-ID	2893931-1
ISSN	2305-5847 ; 2305-5839
ISSN (online)	2305-5847
ISSN	2305-5839
DOI	10.21037/atm.2018.10.35
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Article ; Online: Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study.

Brocklebank, Vicky / Walsh, Patrick R / Smith-Jackson, Kate / Hallam, Thomas M / Marchbank, Kevin J / Wilson, Valerie / Bigirumurame, Theophile / Dutt, Tina / Montgomery, Emma K / Malina, Michal / Wong, Edwin K S / Johnson, Sally / Sheerin, Neil S / Kavanagh, David

Blood

2023 Volume 142, Issue 16, Page(s) 1371–1386

Abstract: Historically, the majority of patients with complement-mediated atypical hemolytic uremic syndrome (CaHUS) progress to end-stage kidney disease (ESKD). Single-arm trials of eculizumab with a short follow-up suggested efficacy. We prove, for the first ... ...

Abstract	Historically, the majority of patients with complement-mediated atypical hemolytic uremic syndrome (CaHUS) progress to end-stage kidney disease (ESKD). Single-arm trials of eculizumab with a short follow-up suggested efficacy. We prove, for the first time to our knowledge, in a genotype matched CaHUS cohort that the 5-year cumulative estimate of ESKD-free survival improved from 39.5% in a control cohort to 85.5% in the eculizumab-treated cohort (hazard ratio, 4.95; 95% confidence interval [CI], 2.75-8.90; P = .000; number needed to treat, 2.17 [95% CI, 1.81-2.73]). The outcome of eculizumab treatment is associated with the underlying genotype. Lower serum creatinine, lower platelet count, lower blood pressure, and younger age at presentation as well as shorter time between presentation and the first dose of eculizumab were associated with estimated glomerular filtration rate >60 ml/min at 6 months in multivariate analysis. The rate of meningococcal infection in the treated cohort was 550 times greater than the background rate in the general population. The relapse rate upon eculizumab withdrawal was 1 per 9.5 person years for patients with a pathogenic mutation and 1 per 10.8 person years for those with a variant of uncertain significance. No relapses were recorded in 67.3 person years off eculizumab in those with no rare genetic variants. Eculizumab was restarted in 6 individuals with functioning kidneys in whom it had been stopped, with no individual progressing to ESKD. We demonstrated that biallelic pathogenic mutations in RNA-processing genes, including EXOSC3, encoding an essential part of the RNA exosome, cause eculizumab nonresponsive aHUS. Recessive HSD11B2 mutations causing apparent mineralocorticoid excess may also present with thrombotic microangiopathy.
MeSH term(s)	Humans ; Child, Preschool ; Atypical Hemolytic Uremic Syndrome/drug therapy ; Atypical Hemolytic Uremic Syndrome/genetics ; Platelet Count ; Complement System Proteins ; Thrombotic Microangiopathies ; Cohort Studies ; Kidney Failure, Chronic/genetics
Chemical Substances	Complement System Proteins (9007-36-7)
Language	English
Publishing date	2023-06-28
Publishing country	United States
Document type	Observational Study ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood.2022018833
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Article: Determining the Potential of DNA Damage Response (DDR) Inhibitors in Cervical Cancer Therapy.

Saha, Santu / Rundle, Stuart / Kotsopoulos, Ioannis C / Begbie, Jacob / Howarth, Rachel / Pappworth, Isabel Y / Mukhopadhyay, Asima / Kucukmetin, Ali / Marchbank, Kevin J / Curtin, Nicola

Cancers

2022 Volume 14, Issue 17

Abstract: Cisplatin-based chemo-radiotherapy (CRT) is the standard treatment for advanced cervical cancer (CC) but the response rate is poor (46-72%) and cisplatin is nephrotoxic. Therefore, better treatment of CC is urgently needed. We have directly compared, for ...

Abstract	Cisplatin-based chemo-radiotherapy (CRT) is the standard treatment for advanced cervical cancer (CC) but the response rate is poor (46-72%) and cisplatin is nephrotoxic. Therefore, better treatment of CC is urgently needed. We have directly compared, for the first time, the cytotoxicity of four DDR inhibitors (rucaparib/PARPi, VE-821/ATRi, PF-477736/CHK1i and MK-1775/WEE1i) as single agents, and in combination with cisplatin and radiotherapy (RT) in a panel of CC cells. All inhibitors alone caused concentration-dependent cytotoxicity. Low ATM and DNA-PKcs levels were associated with greater VE-821 cytotoxicity. Cisplatin induced ATR, CHK1 and WEE1 activity in all of the cell lines. Cisplatin only activated PARP in S-phase cells, but RT activated PARP in the entire population. Rucaparib was the most potent radiosensitiser and VE-821 was the most potent chemosensitiser. VE-821, PF-47736 and MK-1775 attenuated cisplatin-induced S-phase arrest but tended to increase G2 phase accumulation. In mice, cisplatin-induced acute kidney injury was associated with oxidative stress and PARP activation and was prevented by rucaparib. Therefore, while all inhibitors investigated may increase the efficacy of CRT, the greatest clinical potential of rucaparib may be in limiting kidney damage, which is dose-limiting.
Language	English
Publishing date	2022-09-01
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527080-1
ISSN	2072-6694
ISSN	2072-6694
DOI	10.3390/cancers14174288
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Article ; Online: Murine Factor H Co-Produced in Yeast With Protein Disulfide Isomerase Ameliorated C3 Dysregulation in Factor H-Deficient Mice.

Kerr, Heather / Herbert, Andrew P / Makou, Elisavet / Abramczyk, Dariusz / Malik, Talat H / Lomax-Browne, Hannah / Yang, Yi / Pappworth, Isabel Y / Denton, Harriet / Richards, Anna / Marchbank, Kevin J / Pickering, Matthew C / Barlow, Paul N

Frontiers in immunology

2021 Volume 12, Page(s) 681098

Abstract: Recombinant human factor H (hFH) has potential for treating diseases linked to aberrant complement regulation including C3 glomerulopathy (C3G) and dry age-related macular degeneration. Murine FH (mFH), produced in the same host, is useful for pre- ... ...

Abstract	Recombinant human factor H (hFH) has potential for treating diseases linked to aberrant complement regulation including C3 glomerulopathy (C3G) and dry age-related macular degeneration. Murine FH (mFH), produced in the same host, is useful for pre-clinical investigations in mouse models of disease. An abundance of FH in plasma suggests high doses, and hence microbial production, will be needed. Previously,
MeSH term(s)	Animals ; Complement C3/immunology ; Complement C3/metabolism ; Complement Factor H/biosynthesis ; Complement Factor H/deficiency ; Gene Expression ; Immunomodulation ; Mice ; Mice, Knockout ; Protein Disulfide-Isomerases/metabolism ; Recombinant Proteins/chemistry ; Recombinant Proteins/genetics ; Recombinant Proteins/isolation & purification ; Recombinant Proteins/metabolism ; Yeasts/genetics ; Yeasts/metabolism
Chemical Substances	Complement C3 ; Recombinant Proteins ; Complement Factor H (80295-65-4) ; Protein Disulfide-Isomerases (EC 5.3.4.1)
Language	English
Publishing date	2021-05-12
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.2021.681098
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Article ; Online: Rare Genetic Variants in Complement Factor I Lead to Low FI Plasma Levels Resulting in Increased Risk of Age-Related Macular Degeneration.

Hallam, Thomas M / Marchbank, Kevin J / Harris, Claire L / Osmond, Clive / Shuttleworth, Victoria G / Griffiths, Helen / Cree, Angela J / Kavanagh, David / Lotery, Andrew J

Investigative ophthalmology & visual science

2020 Volume 61, Issue 6, Page(s) 18

Abstract: Purpose: Rare genetic variants in complement factor I (CFI) that cause low systemic levels of the protein (FI) have been reported as a strong risk factor for advanced age-related macular degeneration (AMD). This study set out to replicate these findings. ...

Abstract	Purpose: Rare genetic variants in complement factor I (CFI) that cause low systemic levels of the protein (FI) have been reported as a strong risk factor for advanced age-related macular degeneration (AMD). This study set out to replicate these findings. Methods: FI levels were measured by sandwich ELISA in an independent cohort of 276 patients with AMD and 205 elderly controls. Single-nucleotide polymorphism genotyping and Sanger sequencing were used to assess genetic variability. Results: The median FI level was significantly lower in those individuals with AMD and a rare CFI variant (28.3 µg/mL) compared to those with AMD without a rare CFI variant (38.8 µg/mL, P = 0.004) or the control population with (41.7 µg/mL, P = 0.0085) or without (41.5 µg/mL, P < 0.0001) a rare CFI variant. Thirty-six percent of patients with AMD with a rare CFI variant had levels below the fifth percentile, compared to 6% in controls with CFI variants. Multiple regression analyses revealed a decreased FI level associated with a rare CFI variant was a risk factor for AMD (early or late AMD: odds ratio [OR] 12.05, P = 0.03; early AMD: OR 30.3, P = 0.02; late AMD: OR 10.64, P < 0.01). Additionally, measurement of FI in aqueous humor revealed a large FI concentration gradient between systemic circulation and the eye (∼286-fold). Conclusions: Rare genetic variants in CFI causing low systemic FI levels are strongly associated with AMD. The impermeability of the Bruch's membrane to FI will have implications for therapeutic replacement of FI in individuals with CFI variants and low FI levels at risk of AMD.
MeSH term(s)	Aged ; Aged, 80 and over ; Biomarkers ; Case-Control Studies ; Complement Factor H/genetics ; Complement Factor I/genetics ; Complement Factor I/metabolism ; Eye Proteins/genetics ; Female ; Genetic Predisposition to Disease/genetics ; Genetic Testing ; Genetic Variation ; Genotyping Techniques ; Humans ; Macular Degeneration/blood ; Macular Degeneration/genetics ; Male ; Middle Aged ; Polymorphism, Single Nucleotide ; Proportional Hazards Models ; Proteins/genetics ; Receptors, TNF-Related Apoptosis-Inducing Ligand/genetics ; Retrospective Studies ; Risk Factors ; Vascular Endothelial Growth Factor A/genetics
Chemical Substances	ARMS2 protein, human ; Biomarkers ; CFH protein, human ; Eye Proteins ; Proteins ; Receptors, TNF-Related Apoptosis-Inducing Ligand ; TNFRSF10A protein, human ; VEGFA protein, human ; Vascular Endothelial Growth Factor A ; Complement Factor H (80295-65-4) ; CFI protein, human (EC 3.4.21.45) ; Complement Factor I (EC 3.4.21.45)
Language	English
Publishing date	2020-06-25
Publishing country	United States
Document type	Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
ZDB-ID	391794-0
ISSN	1552-5783 ; 0146-0404
ISSN (online)	1552-5783
ISSN	0146-0404
DOI	10.1167/iovs.61.6.18
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Article ; Online: Successful simultaneous liver-kidney transplantation for renal failure associated with hereditary complement C3 deficiency.

Nayagam, Jeremy S / McGrath, Samuel / Montasser, Mahmoud / Delaney, Michael / Cairns, Tom D / Marchbank, Kevin J / Denton, Harriet / Yang, Yi / Sacks, Steven H / Cook, H Terry / Shah, Sapna / Heaton, Nigel / Pickering, Matthew C / Suddle, Abid

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons

2020 Volume 20, Issue 8, Page(s) 2260–2263

Abstract: Hereditary complement C3 deficiency is associated with recurrent bacterial infections and proliferative glomerulonephritis. We describe a case of an adult with complete deficiency of complement C3 due to homozygous mutations in C3 gene: c.1811delT ( ... ...

Abstract	Hereditary complement C3 deficiency is associated with recurrent bacterial infections and proliferative glomerulonephritis. We describe a case of an adult with complete deficiency of complement C3 due to homozygous mutations in C3 gene: c.1811delT (Val604Glyfs*2), recurrent bacterial infections, crescentic glomerulonephritis, and end-stage renal failure. Following isolated kidney transplantation he would remain C3 deficient with a similar, or increased, risk of infections and glomerulonephritis. As C3 is predominantly synthesized in the liver, with a small proportion of C3 monocyte derived and kidney derived, he proceeded to simultaneous liver-kidney transplantation. The procedure has been successful with restoration of his circulating C3 levels, normal liver and kidney function at 26 months of follow-up. Simultaneous liver-kidney transplant is a viable option to be considered in this rare setting.
MeSH term(s)	Adult ; Complement C3/genetics ; Glomerulonephritis ; Humans ; Kidney ; Kidney Failure, Chronic/surgery ; Kidney Transplantation ; Liver ; Male
Chemical Substances	Complement C3
Language	English
Publishing date	2020-02-06
Publishing country	United States
Document type	Case Reports
ZDB-ID	2060594-8
ISSN	1600-6143 ; 1600-6135
ISSN (online)	1600-6143
ISSN	1600-6135
DOI	10.1111/ajt.15785
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