Purification and functional characterization of factor I

Methods in Molecular Biology

Volumn 1100, Issue , 2014, Pages 177-188

  Nilsson, Sara C ^a   Blom, Anna M ^a  

^a LUND UNIVERSITY   (Sweden)

Author keywords

Atypical hemolytic uremic syndrome; Complement inhibitor; Factor I

Indexed keywords

FIBRINOGEN; MONOCLONAL ANTIBODY;

AFFINITY CHROMATOGRAPHY; ANION EXCHANGE CHROMATOGRAPHY; ARTICLE; PRIORITY JOURNAL; PROTEIN DETERMINATION; PROTEIN FUNCTION; PROTEIN PURIFICATION;

BLOTTING, WESTERN; CHROMATOGRAPHY, AFFINITY; ELECTROPHORESIS, POLYACRYLAMIDE GEL; ENZYME-LINKED IMMUNOSORBENT ASSAY; FIBRINOGEN; HUMANS;

EID: 84934443229     PISSN: 10643745     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-1-62703-724-2_15     Document Type: Article

References (15)

1. Morris KM, Aden DP, Knowles BB et al (1982) Complement biosynthesis by the human hepatoma-derived cell line HepG2. J Clin Invest 70:906-913
2. Goldberger G, Arnaout MA, Aden D et al (1984) Biosynthesis and postsynthetic processing of human C3b/C4b inactivator (factor I) in three hepatoma cell lines. J Biol Chem 259:6492-6497
3. Nilsson SC, Karpman D, Vaziri-Sani F et al (2007) A mutation in factor I that is associated with atypical hemolytic uremic syndrome does not affect the function of factor I in complement regulation. Mol Immunol 44:1835-1844
4. Ullman CG, Chamberlain D, Ansari A et al (1998) Human complement factor I: its expression by insect cells and its biochemical and structural characterisation. Mol Immunol 35:503-512
5. Chamberlain D, Ullman CG, Perkins SJ (1998) Possible arrangement of the five domains in human complement factor I as determined by a combination of X-ray and neutron scattering and homology modeling. Biochemistry 37:13918-13929
6. Tsiftsoglou SA, Willis AC, Li P et al (2005) The catalytically active serine protease domain of human complement factor I. Biochemistry 44:6239-6249
7. Roversi P, Johnson S, Caesar JJ et al (2011) Structural basis for complement factor I control and its disease-associated sequence polymorphisms. Proc Natl Acad Sci USA 108:12839-12844
8. Tsiftsoglou SA, Sim RB (2004) Human complement factor I does not require cofactors for cleavage of synthetic substrates. J Immunol 173:367-375
9. Amadei N, Baracho GV, Nudelman V et al (2001) Inherited complete factor I deficiency associated with systemic lupus erythematosus, higher susceptibility to infection and low levels of factor H. Scand J Immunol 53:615-621
10. Nilsson SC, Trouw LA, Renault N et al (2009) Genetic, molecular and functional analyses of complement factor I deficiency. Eur J Immunol 39:310-323
11. Sadallah S, Gudat F, Laissue JA et al (1999) Glomerulonephritis in a patient with complement factor I deficiency. Am J Kidney Dis 33:1153-1157
12. Bienaime F, Dragon-Durey MA, Regnier CH et al (2010) Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome. Kidney Int 77:339-349
13. Esparza-Gordillo J, Jorge EG, Garrido CA et al (2006) Insights into hemolytic uremic syndrome: segregation of three independent predisposition factors in a large, multiple affected pedigree. Mol Immunol 43:1769-1775
14. Kavanagh D, Kemp EJ, Mayland E et al (2005) Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome. J Am Soc Nephrol 16:2150-2155
15. Nilsson SC, Kalchishkova N, Trouw LA et al (2010) Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I. Eur J Immunol 40:172-185