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Original Article

Genetics and Deficiencies of the Soluble Regulatory Proteins of the Complement System

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Pages 65-86 | Received 20 May 1992, Published online: 10 Jul 2009

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Sally S. Twining. (1994) Regulation of Proteolytic Activity in Tissues. Critical Reviews in Biochemistry and Molecular Biology 29:5, pages 315-383.
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Marco Mannes, Arthur Dopler, Markus Huber-Lang & Christoph Q. Schmidt. (2020) Tuning the Functionality by Splicing: Factor H and Its Alternative Splice Variant FHL-1 Share a Gene but Not All Functions. Frontiers in Immunology 11.
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Lubna Kouser, Basudev Paudyal, Anuvinder Kaur, Gudrun Stenbeck, Lucy A. Jones, Suhair M. Abozaid, Cordula M. Stover, Emmanuel Flahaut, Robert B. Sim & Uday Kishore. (2018) Human Properdin Opsonizes Nanoparticles and Triggers a Potent Pro-inflammatory Response by Macrophages without Involving Complement Activation. Frontiers in Immunology 9.
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Haseeb A. Khan, Isra Khan & Yong-kyu Lee. 2018. Fullerens, Graphenes and Nanotubes. Fullerens, Graphenes and Nanotubes 601 630 .
Robert B. Sim. 2013. Handbook of Proteolytic Enzymes. Handbook of Proteolytic Enzymes 2875 2880 .
H. David Pettigrew, Suzanne S. Teuber & M. Eric Gershwin. (2009) Clinical Significance of Complement Deficiencies. Annals of the New York Academy of Sciences 1173:1, pages 108-123.
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M A Friese, J Hellwage, T S Jokiranta, S Meri, H J Müller-Quernheim, H H Peter, H Eibel & P F Zipfel. (2000) Different regulation of factor H and FHL-1/reconectin by inflammatory mediators and expression of the two proteins in rheumatoid arthritis (RA). Clinical and Experimental Immunology 121:2, pages 406-415.
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K. R. Mayilyan, Y. H. Kang, A. W. Dodds & R. B. Sim. 2008. Innate Immunity of Plants, Animals, and Humans. Innate Immunity of Plants, Animals, and Humans 219 236 .
A S Grumach, M F Leitão, V G Arruk, M Kirschfink & A Condino-Neto. (2006) Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family. Clinical and Experimental Immunology 143:2, pages 297-304.
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Syed Asghar, Krisztina Timár & Marcel Pasch. 2004. Skin Immune System. Skin Immune System 327 348 .
Jon D. Stratton & Paul Warwicker. (2002) Successful treatment of factor H‐related haemolytic uraemic syndrome. Nephrology Dialysis Transplantation 17:4, pages 684-685.
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T.Sakari Jokiranta & Seppo Meri. 2002. Wiley Encyclopedia of Molecular Medicine. Wiley Encyclopedia of Molecular Medicine.
C A P Fijen, R G M Bredius, E J Kuijper, T A Out, M De Haas, A P M De Wit, M R Daha & J G J Van De Winkel. (2000) The role of Fc γ receptor polymorphisms and C3 in the immune defence against Neisseria meningitidis in complement-deficient individuals . Clinical and Experimental Immunology 120:2, pages 338-345.
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Jan D Bos, Marcel C Pasch & Syed S Asghar. (2001) Defensins and complement systems from the perspective of skin immunity and autoimmunity. Clinics in Dermatology 19:5, pages 563-572.
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JESSICA CAPRIOLI, PAOLA BETTINAGLIO, PETER F. ZIPFEL, BARBARA AMADEI, ERICA DAINA, SARA GAMBA, CHRISTINE SKERKA, NICOLA MARZILIANO, GIUSEPPE REMUZZI & MARINA NORIS. (2001) The Molecular Basis of Familial Hemolytic Uremic Syndrome: Mutation Analysis of Factor H Gene Reveals a Hot Spot in Short Consensus Repeat 20. Journal of the American Society of Nephrology 12:2, pages 297-307.
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Dean A Male, Rebecca J Ormsby, Shoba Ranganathan, Eleni Giannakis & David L Gordon. (2000) Complement factor H: sequence analysis of 221 kb of human genomic DNA containing the entire fH, fHR-1 and fHR-3 genes. Molecular Immunology 37:1-2, pages 41-52.
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Bernard J. Morley. 2000. The Complement FactsBook. The Complement FactsBook 83 86 .
Lihua Ying, Yitzhak Katz, Menachem Schlesinger, Rivka Carmi, Hanna Shalev, Neena Haider, Gretel Beck, Val C. Sheffield & Daniel Landau. (1999) Complement Factor H Gene Mutation Associated with Autosomal Recessive Atypical Hemolytic Uremic Syndrome. The American Journal of Human Genetics 65:6, pages 1538-1546.
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Manuel A Friese, Jens Hellwage, T.Sakari Jokiranta, Seppo Meri, Hans H Peter, Hermann Eibel & Peter F Zipfel. (1999) FHL-1/reconectin and factor H: two human complement regulators which are encoded by the same gene are differently expressed and regulated. Molecular Immunology 36:13-14, pages 809-818.
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Peter F Zipfel, T.Sakari Jokiranta, Jens Hellwage, Vesa Koistinen & Seppo Meri. (1999) The factor H protein family. Immunopharmacology 42:1-3, pages 53-60.
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Peter F Zipfel & Christine Skerka. (1999) FHL-1/reconectin: a human complement and immune regulator with cell-adhesive function. Immunology Today 20:3, pages 135-140.
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. 1999. Complement Regulatory Proteins. Complement Regulatory Proteins 261 370 .
Heike Kotarsky, Jens Hellwage, Eskil Johnsson, Christine Skerka, Henrik G. Svensson, Gunnar Lindahl, Ulf Sjöbring & Peter F. Zipfel. (1998) Identification of a Domain in Human Factor H and Factor H-Like Protein-1 Required for the Interaction with Streptococcal M Proteins. The Journal of Immunology 160:7, pages 3349-3354.
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Paul Warwicker, Timothy H.J. Goodship, Rosemary L. Donne, Yves Pirson, Anthony Nicholls, Roy M. Ward, Peter Turnpenny & Judith A. Goodship. (1998) Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney International 53:4, pages 836-844.
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Gary S. Coombs, Robert C. Bergstrom, Edwin L. Madison & David R. Corey. (1998) Directing Sequence-specific Proteolysis to New Targets. Journal of Biological Chemistry 273:8, pages 4323-4328.
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Klaus Rother, Gerd O. Till & G. Maria Hänsch. 1998. The Complement System. The Complement System 343 513 .
M.F. Leitão, M.M.S. Vilela, R. Rutz, A.S. Grumach, A. Condino-Neto & M. Kirschfink. (1997) Complement factor I deficiency in a family with recurrent infections. Immunopharmacology 38:1-2, pages 207-213.
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J. L. Williams, D. H. Lester, V. M. Teres, W. Barendse, R. B. Sim & C. J. Soames. (1997) Mapping the bovine factor H gene to Chromosome 16 by SSCP analysis. Mammalian Genome 8:1, pages 77-78.
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Clark D. West. (1994) Nephritic Factors Predispose to Chronic Glomerulonephritis. American Journal of Kidney Diseases 24:6, pages 956-963.
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Peter F. Zipfel & Christine Skerka. (1994) Complement factor H and related proteins: an expanding family of complement-regulatory proteins?. Immunology Today 15:3, pages 121-126.
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