{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,9]],"date-time":"2026-11-09T23:00:56Z","timestamp":1794265256083,"version":"4.9.0"},"reference-count":110,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2008,1,7]],"date-time":"2008-01-07T00:00:00Z","timestamp":1199664000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2008,1,7]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>OTHER ARTICLES PUBLISHED IN THIS TRANSLATIONAL MINI-REVIEW SERIES ON COMPLEMENT FACTOR H<\/jats:p><jats:p>Genetics and disease associations of human complement factor H. Clin Exp Immunol 2008; 151: doi:10.1111\/j.1365-2249.2007.03552.x<\/jats:p><jats:p>Structural and functional correlations for factor H. Clin Exp Immunol 2008; 151: doi:10.1111\/j.1365-2249.2007.03553.x<\/jats:p><jats:p>Therapies of renal diseases associated with complement factor H abnormalities: atypical haemolytic uraemic syndrome and membranoproliferative glomerulonephritis. Clin Exp Immunol 2008; 151: doi:10.1111\/j.1365-2249.2007.03553.x<\/jats:p><jats:p>Factor H is the major regulatory protein of the alternative pathway of complement activation. Abnormalities in factor H have been associated with renal disease, namely glomerulonephritis with C3 deposition including membranoproliferative glomerulonephritis (MPGN) and the atypical haemolytic uraemic syndrome (aHUS). Furthermore, a common factor H polymorphism has been identified as a risk factor for the development of age-related macular degeneration. These associations suggest that alternative pathway dysregulation is a common feature in the pathogenesis of these conditions. However, with respect to factor H-associated renal disease, it is now clear that distinct molecular defects in the protein underlie the pathogenesis of glomerulonephritis and HUS. In this paper we review the associations between human factor H dysfunction and renal disease and explore how observations in both spontaneous and engineered animal models of factor H dysfunction have contributed to our understanding of the pathogenesis of factor H-related renal disease.<\/jats:p>","DOI":"10.1111\/j.1365-2249.2007.03574.x","type":"journal-article","created":{"date-parts":[[2010,7,29]],"date-time":"2010-07-29T12:23:24Z","timestamp":1280406204000},"page":"210-230","source":"Crossref","is-referenced-by-count":160,"title":["Translational Mini-Review Series on Complement Factor H: Renal diseases associated with complement factor H: novel insights from humans and animals"],"prefix":"10.1093","volume":"151","author":[{"given":"M C","family":"Pickering","sequence":"first","affiliation":[{"name":"Molecular Genetics and Rheumatology Section"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H T","family":"Cook","sequence":"additional","affiliation":[{"name":"Department of Histopathology, Faculty of Medicine, Imperial College, Hammersmith Campus, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2008,1,7]]},"reference":[{"key":"2022010406275935200_b1","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1084\/jem.122.2.277","article-title":"Isolation of b1F-globulin from human serum, and its characterisation as the fifth component of complement","volume":"122","author":"Nilsson","year":"1965","journal-title":"J Exp Med"},{"key":"2022010406275935200_b2","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1111\/j.1432-1033.1991.tb16028.x","article-title":"Human complement factor H. Tissue specificity in the expression of three different mRNA species","volume":"198","author":"Schwaeble","year":"1991","journal-title":"Eur J Biochem"},{"key":"2022010406275935200_b3","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1111\/j.1365-2249.1994.tb06033.x","article-title":"Regulation of C3 and factor H synthesis of human glomerular mesangial cells by IL-1 and interferon-gamma","volume":"95","author":"Van Den Dobbelsteen","year":"1994","journal-title":"Clin Exp Immunol"},{"key":"2022010406275935200_b4","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.molimm.2006.07.287","article-title":"The simple design of complement factor H: looks can be deceiving","volume":"44","author":"Alexander","year":"2007","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b5","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/0161-5890(80)90119-4","article-title":"The physiological breakdown of the third component of human complement","volume":"17","author":"Harrison","year":"1980","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b6","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1084\/jem.146.1.257","article-title":"Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum protein beta1H for cleavage of C3b and C4b in solution","volume":"146","author":"Pangburn","year":"1977","journal-title":"J Exp Med"},{"key":"2022010406275935200_b7","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1084\/jem.147.6.1792","article-title":"Interaction of beta1H globulin with cell-bound C3b: quantitative analysis of binding and influence of alternative pathway components on binding","volume":"147","author":"Conrad","year":"1978","journal-title":"J Exp Med"},{"key":"2022010406275935200_b8","doi-asserted-by":"crossref","first-page":"3268","DOI":"10.1073\/pnas.73.9.3268","article-title":"Control of the amplification convertase of complement by the plasma protein beta1H","volume":"73","author":"Weiler","year":"1976","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b9","doi-asserted-by":"crossref","first-page":"2416","DOI":"10.1073\/pnas.75.5.2416","article-title":"Complement C3 convertase: cell surface restriction of beta1H control and generation of restriction on neuraminidase-treated cells","volume":"75","author":"Pangburn","year":"1978","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b10","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1159\/000232106","article-title":"Reaction mechanisms of beta1H globulin","volume":"57","author":"Nagaki","year":"1978","journal-title":"Int Arch Allergy Appl Immunol"},{"key":"2022010406275935200_b11","doi-asserted-by":"crossref","first-page":"977","DOI":"10.4049\/jimmunol.124.2.977","article-title":"Activation of the alternative complement pathway: recognition of surface structures on activators by bound C3b","volume":"124","author":"Pangburn","year":"1980","journal-title":"J Immunol"},{"key":"2022010406275935200_b12","doi-asserted-by":"crossref","first-page":"1971","DOI":"10.1073\/pnas.75.4.1971","article-title":"Regulation by membrane sialic acid of beta1H-dependent decay\u2003\u2212\u2003dissociation of amplification C3 convertase of the alternative complement pathway","volume":"75","author":"Fearon","year":"1978","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b13","doi-asserted-by":"crossref","first-page":"75","DOI":"10.4049\/jimmunol.122.1.75","article-title":"Human alternative complement pathway: membrane-associated sialic acid regulates the competition between B and beta1 H for cell-bound C3b","volume":"122","author":"Kazatchkine","year":"1979","journal-title":"J Immunol"},{"key":"2022010406275935200_b14","doi-asserted-by":"crossref","first-page":"2383","DOI":"10.1002\/eji.1830261017","article-title":"Mapping of the domains required for decay acceleration activity of the human factor H-like protein 1 and factor H","volume":"26","author":"Kuhn","year":"1996","journal-title":"Eur J Immunol"},{"key":"2022010406275935200_b15","doi-asserted-by":"crossref","first-page":"348","DOI":"10.4049\/jimmunol.155.1.348","article-title":"Identification of complement regulatory domains in human factor H","volume":"155","author":"Gordon","year":"1995","journal-title":"J Immunol"},{"key":"2022010406275935200_b16","doi-asserted-by":"crossref","first-page":"10996","DOI":"10.1073\/pnas.93.20.10996","article-title":"Identification of three physically and functionally distinct binding sites for C3b in human complement factor H by deletion mutagenesis","volume":"93","author":"Sharma","year":"1996","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b17","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/S0162-3109(00)80300-8","article-title":"Host recognition and target differentiation by factor H, a regulator of the alternative pathway of complement","volume":"49","author":"Pangburn","year":"2000","journal-title":"Immunopharmacology"},{"key":"2022010406275935200_b18","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1126\/science.1110189","article-title":"Complement factor H polymorphism and age-related macular degeneration","volume":"308","author":"Edwards","year":"2005","journal-title":"Science"},{"key":"2022010406275935200_b19","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1126\/science.1110359","article-title":"Complement factor H variant increases the risk of age-related macular degeneration","volume":"308","author":"Haines","year":"2005","journal-title":"Science"},{"key":"2022010406275935200_b20","doi-asserted-by":"crossref","first-page":"7227","DOI":"10.1073\/pnas.0501536102","article-title":"A common haplotype in the complement regulatory gene factor H (HF1\/CFH) predisposes individuals to age-related macular degeneration","volume":"102","author":"Hageman","year":"2005","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b21","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1126\/science.1109557","article-title":"Complement factor H polymorphism in age-related macular degeneration","volume":"308","author":"Klein","year":"2005","journal-title":"Science"},{"key":"2022010406275935200_b22","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.molimm.2004.02.005","article-title":"The human complement factor H: functional roles, genetic variations and disease associations","volume":"41","author":"Rodriguez de Cordoba","year":"2004","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b23","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1038\/ki.1986.278","article-title":"H deficiency in two brothers with atypical dense intramembranous deposit disease","volume":"30","author":"Levy","year":"1986","journal-title":"Kidney Int"},{"key":"2022010406275935200_b24","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1038\/sj.ki.5000269","article-title":"Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGN II)","volume":"70","author":"Licht","year":"2006","journal-title":"Kidney Int"},{"key":"2022010406275935200_b25","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1042\/bst0150648","article-title":"A familial deficiency of complement factor H","volume":"15","author":"Lopez-Larrea","year":"1987","journal-title":"Biochem Soc Trans"},{"key":"2022010406275935200_b26","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1097\/01.ASN.0000115702.28859.A7","article-title":"Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases","volume":"15","author":"Dragon-Durey","year":"2004","journal-title":"J Am Soc Nephrol"},{"key":"2022010406275935200_b27","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1038\/sj.ki.5000155","article-title":"Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome","volume":"69","author":"Vaziri-Sani","year":"2006","journal-title":"Kidney Int"},{"key":"2022010406275935200_b28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1365-2249.2007.03552.x","article-title":"Translational mini-review series on complement factor H: genetics and disease associations of the human complement factor H","volume":"151","author":"Rodriguez de Cordoba","year":"2008","journal-title":"Clin Exp Immunol"},{"key":"2022010406275935200_b29","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1093\/ndt\/gfg605","article-title":"Factor H deficiency and fibrillary glomerulopathy","volume":"19","author":"Bircan","year":"2004","journal-title":"Nephrol Dial Transplant"},{"key":"2022010406275935200_b30","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1007\/s00467-007-0438-x","article-title":"Complete factor H deficiency-associated atypical hemolytic uremic syndrome in a neonate","volume":"22","author":"Cho","year":"2007","journal-title":"Pediatr Nephrol"},{"key":"2022010406275935200_b31","doi-asserted-by":"crossref","first-page":"2318","DOI":"10.1681\/ASN.V9122318","article-title":"Human complement factor H deficiency associated with hemolytic uremic syndrome","volume":"9","author":"Rougier","year":"1998","journal-title":"J Am Soc Nephrol"},{"key":"2022010406275935200_b32","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1007\/s004670050515","article-title":"Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H","volume":"12","author":"Ohali","year":"1998","journal-title":"Pediatr Nephrol"},{"key":"2022010406275935200_b33","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1046\/j.1365-2249.1996.d01-777.x","article-title":"Heterozygous and homozygous factor H deficiency states in a Dutch family","volume":"105","author":"Fijen","year":"1996","journal-title":"Clin Exp Immunol"},{"key":"2022010406275935200_b34","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1007\/BF00858956","article-title":"Inherited factor H deficiency and collagen type III glomerulopathy","volume":"9","author":"Vogt","year":"1995","journal-title":"Pediatr Nephrol"},{"key":"2022010406275935200_b35","doi-asserted-by":"crossref","first-page":"936","DOI":"10.1016\/S0272-6386(12)81065-1","article-title":"Familial hemolytic\u2013uremic syndrome and homozygous factor H deficiency","volume":"24","author":"Pichette","year":"1994","journal-title":"Am J Kidney Dis"},{"key":"2022010406275935200_b36","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1111\/j.1365-3083.1989.tb02480.x","article-title":"Hereditary, complete deficiency of complement factor H associated with recurrent meningococcal disease","volume":"30","author":"Nielsen","year":"1989","journal-title":"Scand J Immunol"},{"key":"2022010406275935200_b37","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1007\/BF00915156","article-title":"Combined homozygous factor H and heterozygous C2 deficiency in an Italian family","volume":"8","author":"Brai","year":"1988","journal-title":"J Clin Immunol"},{"key":"2022010406275935200_b38","first-page":"110","article-title":"Hypocomplementaemia due to a genetic deficiency of beta 1H globulin","volume":"46","author":"Thompson","year":"1981","journal-title":"Clin Exp Immunol"},{"key":"2022010406275935200_b39","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1007\/s002510050631","article-title":"Molecular basis for factor H and FHL-1 deficiency in an Italian family","volume":"51","author":"Sanchez-Corral","year":"2000","journal-title":"Immunogenetics"},{"key":"2022010406275935200_b40","doi-asserted-by":"crossref","first-page":"11782","DOI":"10.1074\/jbc.274.17.11782","article-title":"Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency","volume":"274","author":"Schmidt","year":"1999","journal-title":"J Biol Chem"},{"key":"2022010406275935200_b41","doi-asserted-by":"crossref","first-page":"25168","DOI":"10.1074\/jbc.272.40.25168","article-title":"Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism","volume":"272","author":"Ault","year":"1997","journal-title":"J Biol Chem"},{"key":"2022010406275935200_b42","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1681\/ASN.V122297","article-title":"The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20","volume":"12","author":"Caprioli","year":"2001","journal-title":"J Am Soc Nephrol"},{"key":"2022010406275935200_b43","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.1086\/302877","article-title":"Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome","volume":"66","author":"Buddles","year":"2000","journal-title":"Am J Hum Genet"},{"key":"2022010406275935200_b44","doi-asserted-by":"crossref","first-page":"1538","DOI":"10.1086\/302673","article-title":"Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome","volume":"65","author":"Ying","year":"1999","journal-title":"Am J Hum Genet"},{"key":"2022010406275935200_b45","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1006\/clim.2000.4878","article-title":"Deficiency of human complement factor I associated with lowered factor H","volume":"96","author":"Naked","year":"2000","journal-title":"Clin Immunol"},{"key":"2022010406275935200_b46","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1038\/ki.1995.82","article-title":"Secondary membranoproliferative glomerulonephritis","volume":"47","author":"Rennke","year":"1995","journal-title":"Kidney Int"},{"key":"2022010406275935200_b47","first-page":"116","article-title":"Alteration singuliere des membranes basales du rein","volume":"68","author":"Berger","year":"1962","journal-title":"J Urol Nephrol"},{"key":"2022010406275935200_b48","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1159\/000180859","article-title":"Dense deposits disease","volume":"19","author":"Droz","year":"1977","journal-title":"Nephron"},{"key":"2022010406275935200_b49","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/ki.1975.32","article-title":"Dense deposit disease: a variant of membranoproliferative glomerulonephritis","volume":"7","author":"Habib","year":"1975","journal-title":"Kidney Int"},{"key":"2022010406275935200_b50","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1038\/ki.1984.71","article-title":"Dense intramembranous deposit disease: new pathologic features","volume":"25","author":"Sibley","year":"1984","journal-title":"Kidney Int"},{"key":"2022010406275935200_b51","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1038\/modpathol.3800773","article-title":"Dense deposit disease is not a membranoproliferative glomerulonephritis","volume":"20","author":"Walker","year":"2007","journal-title":"Mod Pathol"},{"key":"2022010406275935200_b52","doi-asserted-by":"crossref","first-page":"2447","DOI":"10.1681\/ASN.2007030356","article-title":"New approaches to the treatment of dense deposit disease","volume":"18","author":"Smith","year":"2007","journal-title":"J Am Soc Nephrol"},{"key":"2022010406275935200_b53","first-page":"65","article-title":"Membranoproliferative glomerulonephritis with disruption of the glomerular basement membrane","volume":"7","author":"Strife","year":"1977","journal-title":"Clin Nephrol"},{"key":"2022010406275935200_b54","first-page":"474","article-title":"Immunofluorescence studies of dense deposit disease. The presence of railroad tracks and mesangial rings","volume":"40","author":"Kim","year":"1979","journal-title":"Lab Invest"},{"key":"2022010406275935200_b55","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/S0272-6386(87)80089-6","article-title":"Ultrastructural localization of the membrane attack complex of complement in human renal tissues","volume":"9","author":"Falk","year":"1987","journal-title":"Am J Kidney Dis"},{"key":"2022010406275935200_b56","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1126\/science.164.3878.436","article-title":"Serum C\u20323 lytic system in patients with glomerulonephritis","volume":"164","author":"Spitzer","year":"1969","journal-title":"Science"},{"key":"2022010406275935200_b57","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4049\/jimmunol.116.1.1","article-title":"C3 Nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase","volume":"116","author":"Daha","year":"1976","journal-title":"J Immunol"},{"key":"2022010406275935200_b58","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/0002-9343(83)90606-X","article-title":"Idiopathic mesangiocapillary glomerulonephritis. Comparison of types I and II in children and adults and long-term prognosis","volume":"74","author":"Cameron","year":"1983","journal-title":"Am J Med"},{"key":"2022010406275935200_b59","first-page":"240","article-title":"Biological significance of the C3 nephritic factor in membranoproliferative glomerulonephritis","volume":"18","author":"Schena","year":"1982","journal-title":"Clin Nephrol"},{"key":"2022010406275935200_b60","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1056\/NEJM197602262940902","article-title":"The complement abnormalities of lipodystrophy","volume":"294","author":"Sissons","year":"1976","journal-title":"N Engl J Med"},{"key":"2022010406275935200_b61","first-page":"253","article-title":"C3 nephritic factor and hypocomplementaemia in a clinically healthy individual","volume":"54","author":"Gewurz","year":"1983","journal-title":"Clin Exp Immunol"},{"key":"2022010406275935200_b62","first-page":"73","article-title":"Mesangiocapillary glomerulonephritis with dense \u2018deposits\u2019 in the basement membranes of the kidney","volume":"5","author":"Vargas","year":"1976","journal-title":"Clin Nephrol"},{"key":"2022010406275935200_b63","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1038\/ki.1979.51","article-title":"Recurrence of dense deposits in transplanted kidney: II. Serum complement and nephritic factor profiles","volume":"15","author":"Leibowitch","year":"1979","journal-title":"Kidney Int"},{"key":"2022010406275935200_b64","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1681\/ASN.2005020175","article-title":"Recurrence of membranoproliferative glomerulonephritis type II in renal allografts: the North American Pediatric Renal Transplant Cooperative Study experience","volume":"16","author":"Braun","year":"2005","journal-title":"J Am Soc Nephrol"},{"key":"2022010406275935200_b65","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1038\/ki.1979.50","article-title":"Recurrence of dense deposits in transplanted kidneys: I. Sequential survey of the lesions","volume":"15","author":"Droz","year":"1979","journal-title":"Kidney Int"},{"key":"2022010406275935200_b66","first-page":"368","article-title":"Recurrence of an original glomerular lesion in three renal allografts","volume":"3","author":"Galle","year":"1971","journal-title":"Transplant Proc"},{"key":"2022010406275935200_b67","doi-asserted-by":"crossref","first-page":"1723","DOI":"10.1093\/ndt\/14.7.1723","article-title":"Renal transplantation in patients with dense deposit disease: morphological characteristics of recurrent disease and clinical outcome","volume":"14","author":"Andresdottir","year":"1999","journal-title":"Nephrol Dial Transplant"},{"key":"2022010406275935200_b68","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1084\/jem.175.4.939","article-title":"Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis","volume":"175","author":"Meri","year":"1992","journal-title":"J Exp Med"},{"key":"2022010406275935200_b69","doi-asserted-by":"crossref","first-page":"4590","DOI":"10.4049\/jimmunol.163.8.4590","article-title":"Nephritogenic lambda light chain dimer: a unique human miniautoantibody against complement factor H","volume":"163","author":"Jokiranta","year":"1999","journal-title":"J Immunol"},{"key":"2022010406275935200_b70","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1159\/000167525","article-title":"Hypocomplementemic glomerulonephritis in an infant and mother. Evidence for an abnormal form of C3","volume":"7","author":"Linshaw","year":"1987","journal-title":"Am J Nephrol"},{"key":"2022010406275935200_b71","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1038\/ki.1983.89","article-title":"An inherited defect in the C3 convertase, C3b,Bb, associated with glomerulonephritis","volume":"23","author":"Marder","year":"1983","journal-title":"Kidney Int"},{"key":"2022010406275935200_b72","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1136\/jmg.2006.045328","article-title":"Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome","volume":"44","author":"Servais","year":"2007","journal-title":"J Med Genet"},{"key":"2022010406275935200_b73","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1038\/ki.1978.69","article-title":"Prolonged complement activation in mice","volume":"13","author":"Simpson","year":"1978","journal-title":"Kidney Int"},{"key":"2022010406275935200_b74","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1038\/ki.1974.94","article-title":"Lack of nephritogenicity of systemic activation of the alternate complement pathway","volume":"6","author":"Verroust","year":"1974","journal-title":"Kidney Int"},{"key":"2022010406275935200_b75","first-page":"1356","article-title":"Extensive complement activation in hereditary porcine membranoproliferative glomerulonephritis type II (porcine dense deposit disease)","volume":"143","author":"Jansen","year":"1993","journal-title":"Am J Pathol"},{"key":"2022010406275935200_b76","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1046\/j.1523-1755.1998.00765.x","article-title":"In situ complement activation in porcine membranoproliferative glomerulonephritis type II","volume":"53","author":"Jansen","year":"1998","journal-title":"Kidney Int"},{"key":"2022010406275935200_b77","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.1172\/JCI117751","article-title":"Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency","volume":"95","author":"Hogasen","year":"1995","journal-title":"J Clin Invest"},{"key":"2022010406275935200_b78","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1136\/vr.137.10.240","article-title":"Porcine membranoproliferative glomerulonephritis type II: an autosomal recessive deficiency of factor H","volume":"137","author":"Jansen","year":"1995","journal-title":"Vet Rec"},{"key":"2022010406275935200_b79","doi-asserted-by":"crossref","first-page":"2027","DOI":"10.1016\/S0002-9440(10)64481-1","article-title":"The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor H coding sequence block protein secretion","volume":"161","author":"Hegasy","year":"2002","journal-title":"Am J Pathol"},{"key":"2022010406275935200_b80","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1136\/vr.140.15.392","article-title":"Eradication of porcine factor H deficiency in Norway","volume":"140","author":"Hogasen","year":"1997","journal-title":"Vet Rec"},{"key":"2022010406275935200_b81","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1038\/ng912","article-title":"Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H","volume":"31","author":"Pickering","year":"2002","journal-title":"Nat Genet"},{"key":"2022010406275935200_b82","doi-asserted-by":"crossref","first-page":"9649","DOI":"10.1073\/pnas.0601094103","article-title":"Prevention of C5 activation ameliorates spontaneous and experimental glomerulonephritis in factor H-deficient mice","volume":"103","author":"Pickering","year":"2006","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b83","doi-asserted-by":"crossref","first-page":"3924","DOI":"10.1016\/j.molimm.2007.06.041","article-title":"Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice","volume":"44","author":"Rose","year":"2007","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b84","first-page":"385","article-title":"Hereditary complement factor I deficiency","volume":"87","author":"Vyse","year":"1994","journal-title":"Q J Med"},{"key":"2022010406275935200_b85","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1046\/j.1365-3083.2001.00931.x","article-title":"Inherited complete factor I deficiency associated with systemic lupus erythematosus, higher susceptibility to infection and low levels of factor H","volume":"53","author":"Amadei","year":"2001","journal-title":"Scand J Immunol"},{"key":"2022010406275935200_b86","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1080\/00365540510034536","article-title":"Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis","volume":"37","author":"Genel","year":"2005","journal-title":"Scand J Infect Dis"},{"key":"2022010406275935200_b87","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1016\/S0272-6386(99)70155-1","article-title":"Glomerulonephritis in a patient with complement factor I deficiency","volume":"33","author":"Sadallah","year":"1999","journal-title":"Am J Kidney Dis"},{"key":"2022010406275935200_b88","first-page":"348","article-title":"Complement C3 gene expression and regulation in human glomerular epithelial cells","volume":"79","author":"Sacks","year":"1993","journal-title":"Immunology"},{"key":"2022010406275935200_b89","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1111\/j.1365-2249.1993.tb08193.x","article-title":"C3 and C4 gene expression and interferon-gamma-mediated regulation in human glomerular mesangial cells","volume":"93","author":"Sacks","year":"1993","journal-title":"Clin Exp Immunol"},{"key":"2022010406275935200_b90","doi-asserted-by":"crossref","first-page":"1120","DOI":"10.1053\/ajkd.2001.24511","article-title":"Composition of nephritic factor-generated glomerular deposits in membranoproliferative glomerulonephritis type 2","volume":"37","author":"West","year":"2001","journal-title":"Am J Kidney Dis"},{"key":"2022010406275935200_b91","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1136\/jmg.40.9.676","article-title":"Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries","volume":"40","author":"Neumann","year":"2003","journal-title":"J Med Genet"},{"key":"2022010406275935200_b92","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1111\/j.1523-1755.1998.00824.x","article-title":"Genetic studies into inherited and sporadic hemolytic uremic syndrome","volume":"53","author":"Warwicker","year":"1998","journal-title":"Kidney Int"},{"key":"2022010406275935200_b93","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1086\/318203","article-title":"Factor H mutations in hemolytic uremic syndrome cluster in exons 18\u201320, a domain important for host cell recognition","volume":"68","author":"Richards","year":"2001","journal-title":"Am J Hum Genet"},{"key":"2022010406275935200_b94","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1086\/318201","article-title":"Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome","volume":"68","author":"Perez-Caballero","year":"2001","journal-title":"Am J Hum Genet"},{"key":"2022010406275935200_b95","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1136\/jmg.2004.019083","article-title":"Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome","volume":"41","author":"Fremeaux-Bacchi","year":"2004","journal-title":"J Med Genet"},{"key":"2022010406275935200_b96","doi-asserted-by":"crossref","first-page":"1542","DOI":"10.1016\/S0140-6736(03)14742-3","article-title":"Familial haemolytic uraemic syndrome and an MCP mutation","volume":"362","author":"Noris","year":"2003","journal-title":"Lancet"},{"key":"2022010406275935200_b97","doi-asserted-by":"crossref","first-page":"12966","DOI":"10.1073\/pnas.2135497100","article-title":"Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome","volume":"100","author":"Richards","year":"2003","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b98","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1073\/pnas.0603420103","article-title":"Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome","volume":"104","author":"Goicoechea de Jorge","year":"2007","journal-title":"Proc Natl Acad Sci USA"},{"key":"2022010406275935200_b99","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1172\/JCI16651","article-title":"Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome","volume":"111","author":"Manuelian","year":"2003","journal-title":"J Clin Invest"},{"key":"2022010406275935200_b100","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1086\/344515","article-title":"Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome","volume":"71","author":"Sanchez-Corral","year":"2002","journal-title":"Am J Hum Genet"},{"key":"2022010406275935200_b101","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.molimm.2004.01.003","article-title":"Functional analysis in serum from atypical hemolytic uremic syndrome patients reveals impaired protection of host cells associated with mutations in factor H","volume":"41","author":"Sanchez-Corral","year":"2004","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b102","doi-asserted-by":"crossref","first-page":"1249","DOI":"10.1084\/jem.20070301","article-title":"Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains","volume":"204","author":"Pickering","year":"2007","journal-title":"J Exp Med"},{"key":"2022010406275935200_b103","doi-asserted-by":"crossref","first-page":"972","DOI":"10.1016\/j.molimm.2005.05.011","article-title":"Comparison of surface recognition and C3b binding properties of mouse and human complement factor H","volume":"43","author":"Cheng","year":"2006","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b104","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1182\/blood-2005-10-007252","article-title":"Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome","volume":"108","author":"Caprioli","year":"2006","journal-title":"Blood"},{"key":"2022010406275935200_b105","doi-asserted-by":"crossref","first-page":"E22","DOI":"10.1053\/ajkd.2002.36938","article-title":"Recurrence of hemolytic uremic syndrome after live related renal transplantation associated with subsequent de novo disease in the donor","volume":"40","author":"Donne","year":"2002","journal-title":"Am J Kidney Dis"},{"key":"2022010406275935200_b106","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1093\/hmg\/ddi066","article-title":"Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32","volume":"14","author":"Esparza-Gordillo","year":"2005","journal-title":"Hum Mol Genet"},{"key":"2022010406275935200_b107","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1016\/j.molimm.2005.11.008","article-title":"Insights into hemolytic uremic syndrome: segregation of three independent predisposition factors in a large, multiple affected pedigree","volume":"43","author":"Esparza-Gordillo","year":"2006","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b108","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1371\/journal.pgen.0030041","article-title":"Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome","volume":"43","author":"Zipfel","year":"2007","journal-title":"PLoS Genet"},{"key":"2022010406275935200_b109","doi-asserted-by":"crossref","first-page":"3924","DOI":"10.1016\/j.molimm.2007.06.040","article-title":"Factor H autoantibodies in atypical hemolytic uraemic syndrome correlate with CFHR1\/CFHR3 deficiency and affect recognition functions","volume":"44","author":"Jozsi","year":"2007","journal-title":"Mol Immunol"},{"key":"2022010406275935200_b110","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1182\/blood-2007-02-071472","article-title":"Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome","volume":"110","author":"Jozsi","year":"2007","journal-title":"Blood"}],"container-title":["Clinical and Experimental Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2249.2007.03574.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2249.2007.03574.x","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/151\/2\/210\/42038714\/j.1365-2249.2007.03574.x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/cei\/article-pdf\/151\/2\/210\/42038714\/j.1365-2249.2007.03574.x.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,23]],"date-time":"2025-02-23T15:47:23Z","timestamp":1740325643000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/cei\/article\/151\/2\/210\/6457622"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,1,7]]},"references-count":110,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2008,1,7]]},"published-print":{"date-parts":[[2008,1,7]]}},"URL":"https:\/\/doi.org\/10.1111\/j.1365-2249.2007.03574.x","relation":{},"ISSN":["0009-9104","1365-2249"],"issn-type":[{"value":"0009-9104","type":"print"},{"value":"1365-2249","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2008,2]]},"published":{"date-parts":[[2008,1,7]]}}}