Publications
146 results found
Thurman JM, Kulik L, Orth H, et al., 2013, Detection of complement activation using monoclonal antibodies against C3d, JOURNAL OF CLINICAL INVESTIGATION, Vol: 123, Pages: 2218-2230, ISSN: 0021-9738
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- Citations: 61
Banda NK, Mehta G, Ferreira VP, et al., 2013, Essential Role of Surface-Bound Complement Factor H in Controlling Immune Complex-Induced Arthritis, JOURNAL OF IMMUNOLOGY, Vol: 190, Pages: 3560-3569, ISSN: 0022-1767
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- Citations: 23
de Jorge EG, Caesar JJE, Malik TH, et al., 2013, Dimerization of complement factor H-related proteins modulates complement activation in vivo, PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, Vol: 110, Pages: 4685-4690, ISSN: 0027-8424
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- Citations: 197
Fossati-Jimack L, Ling GS, Cortini A, et al., 2013, Phagocytosis Is the Main CR3-Mediated Function Affected by the Lupus-Associated Variant of CD11b in Human Myeloid Cells, PLOS ONE, Vol: 8, ISSN: 1932-6203
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- Citations: 50
Johnson S, Tan L, van der Veen S, et al., 2013, Correction: Design and Evaluation of Meningococcal Vaccines through Structure-Based Modification of Host and Pathogen Molecules., PLoS Pathog, Vol: 9
[This corrects the article on p. e1002981 in vol. 8.].
Ruseva MM, Vernon KA, Lesher AM, et al., 2013, Loss of Properdin Exacerbates C3 Glomerulopathy Resulting from Factor H Deficiency, JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, Vol: 24, Pages: 43-52, ISSN: 1046-6673
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- Citations: 58
Johnson S, Tan L, van der Veen S, et al., 2012, Design and Evaluation of Meningococcal Vaccines through Structure-Based Modification of Host and Pathogen Molecules, PLOS PATHOGENS, Vol: 8, ISSN: 1553-7366
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- Citations: 49
Westra D, Vernon KA, Volokhina EB, et al., 2012, Atypical hemolytic uremic syndrome and genetic aberrations in the complement factor H-related 5 gene, Journal of Human Genetics, Vol: 57, Pages: 459-464, ISSN: 1434-5161
Atypical hemolytic uremic syndrome (aHUS) is a severe renal disorder that is associated with mutations in genes encoding proteins of the alternative complement pathway. Previously, we identified pathogenic variations in genes encoding complement regulators (CFH, CFI and MCP) in our aHUS cohort. In this study, we screened for mutations in the alternative pathway regulator CFHR5 in 65 aHUS patients by means of PCR on genomic DNA and sequence analysis. Potential pathogenicity of genetic alterations was determined by published data on CFHR5 variants, evolutionary conservation and in silico mutation prediction programs. Detection of serum CFHR5 was performed by western blot analysis and enzyme-linked immunosorbent assay. A potentially pathogenic sequence variation was found in CFHR5 in three patients (4.6%). All variations were located in short consensus repeats that might be involved in binding to C3b, heparin or C-reactive protein. The identified CFHR5 mutations require functional studies to determine their relevance to aHUS, but they might be candidates for an altered genetic profile predisposing to the disease.
Malik TH, Lavin PJ, de Jorge EG, et al., 2012, A Hybrid <i>CFHR3</i>-<i>1</i> Gene Causes Familial C3 Glomerulopathy, JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, Vol: 23, Pages: 1155-1160, ISSN: 1046-6673
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- Citations: 93
Vernon KA, de Jorge EG, Hall AE, et al., 2012, Acute Presentation and Persistent Glomerulonephritis Following Streptococcal Infection in a Patient With Heterozygous Complement Factor H-Related Protein 5 Deficiency, AMERICAN JOURNAL OF KIDNEY DISEASES, Vol: 60, Pages: 121-125, ISSN: 0272-6386
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- Citations: 70
Mehta P, Patel L, Roncaroli F, et al., 2012, Painful myositis in the anti-synthetase syndrome with anti-PL12 antibodies, RHEUMATOLOGY INTERNATIONAL, Vol: 32, Pages: 825-827, ISSN: 0172-8172
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- Citations: 2
Sargsyan SA, Serkova NJ, Renner B, et al., 2012, Detection of glomerular complement C3 fragments by magnetic resonance imaging in murine lupus nephritis, KIDNEY INTERNATIONAL, Vol: 81, Pages: 152-159, ISSN: 0085-2538
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- Citations: 38
Menikou S, Patel MP, Rose KL, et al., 2012, Relationship between complotype and severity of reported systemic allergic reactions to peanut, Journal of Allergy and Clinical Immunology, Vol: 129, Pages: 1398-1401
Gale DP, Pickering MC, 2011, Regulating complement in the kidney: insights from CFHR5 nephropathy, DISEASE MODELS & MECHANISMS, Vol: 4, Pages: 721-726, ISSN: 1754-8403
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- Citations: 23
Smith RJH, Harris CL, Pickering MC, 2011, Dense deposit disease, MOLECULAR IMMUNOLOGY, Vol: 48, Pages: 1604-1610, ISSN: 0161-5890
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- Citations: 62
Renner B, Ferreira VP, Cortes C, et al., 2011, Binding of factor H to tubular epithelial cells limits interstitial complement activation in ischemic injury, KIDNEY INTERNATIONAL, Vol: 80, Pages: 166-174, ISSN: 0085-2538
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- Citations: 45
Vernon KA, Gale DP, de Jorge EG, et al., 2011, Recurrence of complement factor H-related protein 5 nephropathy in a renal transplant, American Journal of Transplantation, Vol: 11, Pages: 152-155, ISSN: 1600-6135
Complement factor H‐related protein 5 (CFHR5) nephropathy is a familial renal disease endemic in Cyprus. It is characterized by persistent microscopic hematuria, synpharyngitic macroscopic hematuria and progressive renal impairment. Isolated glomerular accumulation of complement component 3 (C3) is typical with variable degrees of glomerular inflammation. Affected individuals have a heterozygous internal duplication in the CFHR5 gene, although the mechanism through which this mutation results in renal disease is not understood. Notably, the risk of progressive renal failure in this condition is higher in males than females. We report the first documented case of recurrence of CFHR5 nephropathy in a renal transplant in a 53‐year‐old Cypriot male. Strikingly, histological changes of CFHR5 nephropathy were evident in the donor kidney 46 days post‐transplantation. This unique case demonstrates that renal‐derived CFHR5 protein cannot prevent the development of CFHR5 nephropathy.
de Jorge EG, Macor P, Paixao-Cavalcante D, et al., 2011, The Development of Atypical Hemolytic Uremic Syndrome Depends on Complement C5, JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, Vol: 22, Pages: 137-145, ISSN: 1046-6673
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- Citations: 90
Vernon KA, Pickering MC, Cook HT, 2011, Experimental Models of Membranoproliferative Glomerulonephritis, Including Dense Deposit Disease, EXPERIMENTAL MODELS FOR RENAL DISEASES: PATHOGENESIS AND DIAGNOSIS, Vol: 169, Pages: 198-210, ISSN: 0302-5144
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- Citations: 9
de Jorge EG, Pickering MC, 2010, Atypical hemolytic uremic syndrome: telling the difference between H and Y, KIDNEY INTERNATIONAL, Vol: 78, Pages: 721-723, ISSN: 0085-2538
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- Citations: 5
Gale DP, de Jorge EG, Cook HT, et al., 2010, Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis, LANCET, Vol: 376, Pages: 794-801, ISSN: 0140-6736
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- Citations: 235
Fakhouri F, Fremeaux-Bacchi V, Noel L-H, et al., 2010, C3 glomerulopathy: a new classification, NATURE REVIEWS NEPHROLOGY, Vol: 6, Pages: 494-499, ISSN: 1759-5061
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- Citations: 236
Pickering MC, Botto M, 2010, Are anti-C1q antibodies different from other SLE autoantibodies?, NATURE REVIEWS RHEUMATOLOGY, Vol: 6, Pages: 490-493, ISSN: 1759-4790
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- Citations: 38
Fakhouri F, de Jorge EG, Brune F, et al., 2010, Treatment with human complement factor H rapidly reverses renal complement deposition in factor H-deficient mice, KIDNEY INTERNATIONAL, Vol: 78, Pages: 279-286, ISSN: 0085-2538
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- Citations: 73
Mehta P, Norsworthy PJ, Hall AE, et al., 2010, SLE with C1q deficiency treated with fresh frozen plasma: a 10-year experience, RHEUMATOLOGY, Vol: 49, Pages: 823-824, ISSN: 1462-0324
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- Citations: 43
Waters AM, Pappworth I, Marchbank K, et al., 2010, Successful Renal Transplantation in Factor H Autoantibody Associated HUS with CFHR1 and 3 Deficiency and CFH Variant G2850T, AMERICAN JOURNAL OF TRANSPLANTATION, Vol: 10, Pages: 168-172, ISSN: 1600-6135
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- Citations: 30
Leung VWY, Yun S, Botto M, et al., 2009, Decay-Accelerating Factor Suppresses Complement C3 Activation and Retards Atherosclerosis in Low-Density Lipoprotein Receptor-Deficient Mice, AMERICAN JOURNAL OF PATHOLOGY, Vol: 175, Pages: 1757-1767, ISSN: 0002-9440
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- Citations: 37
Mehta P, Fernando MMA, Pickering MC, et al., 2009, Lateral medullary syndrome with anti-neuronal antibodies (anti-Ta/Ma2) in primary Sjogren's syndrome, RHEUMATOLOGY, Vol: 48, Pages: 1174-1176, ISSN: 1462-0324
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- Citations: 3
Botto M, Kirschfink M, Macor P, et al., 2009, Complement in human diseases: Lessons from complement deficiencies, MOLECULAR IMMUNOLOGY, Vol: 46, Pages: 2774-2783, ISSN: 0161-5890
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- Citations: 184
Taylor SRJ, Turner CM, Elliott JI, et al., 2009, P2X<sub>7</sub> Deficiency Attenuates Renal Injury in Experimental Glomerulonephritis, JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, Vol: 20, Pages: 1275-1281, ISSN: 1046-6673
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- Citations: 98
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