Literature DB >> 11429311

Hemolytic uremic syndrome: how do factor H mutants mediate endothelial damage?

P F Zipfel1.   

Abstract

Hemolytic uremic syndrome is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Data from recent genetic analyses reveal a clear association between the complement regulator factor H and the atypical form of this fatal human disease. The clustering of the identified mutations in the C-terminus of factor H identifies a "hot spot" that is central to the pathogenesis of the disease. What are the possible biological and functional consequences of the identified mutations for the disease process and mechanisms of disease progression?

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Year:  2001        PMID： 11429311     DOI： 10.1016/s1471-4906(01)01972-x

Source DB:  PubMed          Journal:  Trends Immunol        ISSN： 1471-4906            Impact factor:   16.687


  12 in total

1.  The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor H coding sequence block protein secretion.

Authors:  Guido A Hegasy; Tamara Manuelian; Kolbjorn Hogasen; Johan H Jansen; Peter F Zipfel
Journal:  Am J Pathol       Date:  2002-12       Impact factor: 4.307

Review 2.  Modern complement analysis.

Authors:  Michael Kirschfink; Tom E Mollnes
Journal:  Clin Diagn Lab Immunol       Date:  2003-11

3.  The C-terminus of complement regulator Factor H mediates target recognition: evidence for a compact conformation of the native protein.

Authors:  M Oppermann; T Manuelian; M Józsi; E Brandt; T S Jokiranta; S Heinen; S Meri; C Skerka; O Götze; P F Zipfel
Journal:  Clin Exp Immunol       Date:  2006-05       Impact factor: 4.330

Review 4.  Viral-derived complement inhibitors: current status and potential role in immunomodulation.

Authors:  Hadi Abou-El-Hassan; Hassan Zaraket
Journal:  Exp Biol Med (Maywood)       Date:  2016-10-26

Review 5.  Complement factor H in AMD: Bridging genetic associations and pathobiology.

Authors:  Christopher B Toomey; Lincoln V Johnson; Catherine Bowes Rickman
Journal:  Prog Retin Eye Res       Date:  2017-09-18       Impact factor: 21.198

6.  Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome.

Authors:  Pilar Sánchez-Corral; David Pérez-Caballero; Olatz Huarte; Ari M Simckes; Elena Goicoechea; Margarita López-Trascasa; Santiago Rodríguez de Córdoba
Journal:  Am J Hum Genet       Date:  2002-11-06       Impact factor: 11.025

7.  Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality.

Authors:  Angela Gerber; Antje H Kirchhoff-Moradpour; Silke Obieglo; Matthias Brandis; Michael Kirschfink; Peter F Zipfel; Judith A Goodship; Lothar B Zimmerhackl
Journal:  Pediatr Nephrol       Date:  2003-06-26       Impact factor: 3.714

Review 8.  Clinical practice. Today's understanding of the haemolytic uraemic syndrome.

Authors:  Johanna Scheiring; Alejandra Rosales; Lothar Bernd Zimmerhackl
Journal:  Eur J Pediatr       Date:  2009-08-26       Impact factor: 3.183

9.  Release of endogenous anti-inflammatory complement regulators FHL-1 and factor H protects synovial fibroblasts during rheumatoid arthritis.

Authors:  M A Friese; T Manuelian; S Junnikkala; J Hellwage; S Meri; H H Peter; D L Gordon; H Eibel; P F Zipfel
Journal:  Clin Exp Immunol       Date:  2003-06       Impact factor: 4.330

10.  Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome.

Authors:  Tamara Manuelian; Jens Hellwage; Seppo Meri; Jessica Caprioli; Marina Noris; Stefan Heinen; Mihaly Jozsi; Hartmut P H Neumann; Giuseppe Remuzzi; Peter F Zipfel
Journal:  J Clin Invest       Date:  2003-04       Impact factor: 14.808
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