Complement Factor H (CFH)


CF-H; FH; FHL1; ARMD4; ARMS1; CFHL3; HF1; HF2; HUS; H Factor 2; Age-Related Maculopathy Susceptibility 1; Adrenomedullin binding protein

Complement Factor H (CFH)

Factor H is a member of the regulators of complement activation family and is a complement control protein. It is a large (155 kilodaltons), soluble glycoprotein that circulates in human plasma (at a concentration of 500–800 micrograms per milliliter). Its main job is to regulate the Alternative Pathway of the complement system, ensuring that the complement system is directed towards pathogens and does not damage host tissue. Factor H regulates complement activation on self cells by possessing both cofactor activity for the Factor I mediated C3b cleavage, and decay accelerating activity against the alternative pathway C3 convertase, C3bBb. Factor H protects self cells from complement activation but not bacteria/viruses, in that it binds to glycosaminoglycans (GAGs) that are present on host cells but not pathogen cell surfaces.

Organism species: Homo sapiens (Human)

ProteinsRPA635Hu01Recombinant Complement Factor H (CFH)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAA635Hu01Polyclonal Antibody to Complement Factor H (CFH)WB, ICC, IHC-P, IHC-F, ELISA
MAA635Hu22Monoclonal Antibody to Complement Factor H (CFH)WB, ICC, IHC-P, IHC-F, ELISA
LAA635Hu71Biotin-Linked Polyclonal Antibody to Complement Factor H (CFH)IHC;WB;ELISA
LAA635Hu81FITC-Linked Polyclonal Antibody to Complement Factor H (CFH)IHC;ICC;IF;WB
Assay KitsSEA635HuELISA Kit for Complement Factor H (CFH)Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

ProteinsRPA635Mu01Recombinant Complement Factor H (CFH)SDS-PAGE; WB; ELISA; IP.
RPA635Mu02Recombinant Complement Factor H (CFH)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAA635Mu01Polyclonal Antibody to Complement Factor H (CFH)WB, ICC, IHC-P, IHC-F, ELISA
PAA635Mu02Polyclonal Antibody to Complement Factor H (CFH)WB, ICC, IHC-P, IHC-F, ELISA
Assay KitsSEA635MuELISA Kit for Complement Factor H (CFH)Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

ProteinsRPA635Ra01Recombinant Complement Factor H (CFH)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAA635Ra01Polyclonal Antibody to Complement Factor H (CFH)WB; ICC; IHC-P; IHC-F; ELISA; IP; IF; FCM.
MAA635Ra21Monoclonal Antibody to Complement Factor H (CFH)WB; ICC; IHC-P; IHC-F; ELISA; IP; IF; FCM.
Assay KitsCEA635RaELISA Kit for Complement Factor H (CFH)Enzyme-linked immunosorbent assay for Antigen Detection
  1. "The complete amino acid sequence of human complement factor H."Biochem. J. 249:593-602(1988) [PubMed] [Europe PMC] [Abstract]
  2. "The DNA sequence and biological annotation of human chromosome 1." Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  4. "Human complement factor H: isolation of cDNA clones and partial cDNA sequence of the 38-kDa tryptic fragment containing the binding site for C3b."Eur. J. Immunol. 16:1351-1355(1986) [PubMed] [Europe PMC] [Abstract]
  5. "Structural analysis of human complement protein H: homology with C4b binding protein, beta 2-glycoprotein I, and the Ba fragment of B2."J. Immunol. 136:3407-3411(1986) [PubMed] [Europe PMC] [Abstract]
  6. "Cloning of the 1.4-kb mRNA species of human complement factor H reveals a novel member of the short consensus repeat family related to the carboxy terminal of the classical 150-kDa molecule."J. Immunol. 146:3190-3196(1991) [PubMed] [Europe PMC] [Abstract]
  7. "Purification and structural studies on the complement-system control protein beta 1H (Factor H)."Biochem. J. 205:285-293(1982) [PubMed] [Europe PMC] [Abstract]
  8. Thesis (1993), Hospital Trias I Pujol, Spain
  9. "Screening for N-glycosylated proteins by liquid chromatography mass spectrometry."Proteomics 4:454-465(2004) [PubMed] [Europe PMC] [Abstract]
  10. "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
  11. "Site-specific N-glycan characterization of human complement factor H."Glycobiology 17:932-944(2007) [PubMed] [Europe PMC] [Abstract]
  12. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
  13. "A strategy for precise and large scale identification of core fucosylated glycoproteins."Mol. Cell. Proteomics 8:913-923(2009) [PubMed] [Europe PMC] [Abstract]
  14. "Enrichment of glycopeptides for glycan structure and attachment site identification."Nat. Methods 6:809-811(2009) [PubMed] [Europe PMC] [Abstract]
  15. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  16. "Three-dimensional structure of a complement control protein module in solution."J. Mol. Biol. 219:717-725(1991) [PubMed] [Europe PMC] [Abstract]
  17. "Solution structure of the fifth repeat of factor H: a second example of the complement control protein module."Biochemistry 31:3626-3634(1992) [PubMed] [Europe PMC] [Abstract]
  18. "Solution structure of a pair of complement modules by nuclear magnetic resonance."J. Mol. Biol. 232:268-284(1993) [PubMed] [Europe PMC] [Abstract]
  19. "Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism."J. Biol. Chem. 272:25168-25175(1997) [PubMed] [Europe PMC] [Abstract]
  20. "Genetic studies into inherited and sporadic hemolytic uremic syndrome."Kidney Int. 53:836-844(1998) [PubMed] [Europe PMC] [Abstract]
  21. "Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome."Am. J. Hum. Genet. 65:1538-1546(1999) [PubMed] [Europe PMC] [Abstract]
  22. "Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome."Am. J. Hum. Genet. 66:1721-1722(2000) [PubMed] [Europe PMC] [Abstract]
  23. "Molecular basis for factor H and FHL-1 deficiency in an Italian family."Immunogenetics 51:366-369(2000) [PubMed] [Europe PMC] [Abstract]
  24. "Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome."Am. J. Hum. Genet. 68:478-484(2001) [PubMed] [Europe PMC] [Abstract]
  25. "Factor H mutations in hemolytic uremic syndrome cluster in exons 18-20, a domain important for host cell recognition."Am. J. Hum. Genet. 68:485-490(2001) [PubMed] [Europe PMC] [Abstract]
  26. "The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20."J. Am. Soc. Nephrol. 12:297-307(2001) [PubMed] [Europe PMC] [Abstract]
  27. "Molecular modelling of the C-terminal domains of factor H of human complement: a correlation between haemolytic uraemic syndrome and a predicted heparin binding site."J. Mol. Biol. 316:217-224(2002) [PubMed] [Europe PMC] [Abstract]
  28. "Combined kidney and liver transplantation for familial haemolytic uraemic syndrome."Lancet 359:1671-1672(2002) [PubMed] [Europe PMC] [Abstract]
  29. "Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease."Hum. Mol. Genet. 12:3385-3395(2003) [PubMed] [Europe PMC] [Abstract]
  30. "Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries."J. Med. Genet. 40:676-681(2003) [PubMed] [Europe PMC] [Abstract]
  31. "Heterozygous and homozygous factor H deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases."J. Am. Soc. Nephrol. 15:787-795(2004) [PubMed] [Europe PMC] [Abstract]
  32. "Strong association of the Y402H variant in complement factor H at 1q32 with susceptibility to age-related macular degeneration."Am. J. Hum. Genet. 77:149-153(2005) [PubMed] [Europe PMC] [Abstract]
  33. "A common haplotype in the complement regulatory gene factor H (HF1/CFH) predisposes individuals to age-related macular degeneration." Proc. Natl. Acad. Sci. U.S.A. 102:7227-7232(2005) [PubMed] [Europe PMC] [Abstract]
  34. "Complement factor H polymorphism in age-related macular degeneration."Science 308:385-389(2005) [PubMed] [Europe PMC] [Abstract]
  35. "Complement factor H variant increases the risk of age-related macular degeneration."Science 308:419-421(2005) [PubMed] [Europe PMC] [Abstract]
  36. "Complement factor H polymorphism and age-related macular degeneration."Science 308:421-424(2005) [PubMed] [Europe PMC] [Abstract]
  37. "Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGN II)."Kidney Int. 70:42-50(2006) [PubMed] [Europe PMC] [Abstract]
  38. "Basal laminar drusen caused by compound heterozygous variants in the CFH gene."Am. J. Hum. Genet. 82:516-523(2008) [PubMed] [Europe PMC] [Abstract]
  39. "Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome."Hum. Mutat. 31:E1445-E1460(2010) [PubMed] [Europe PMC] [Abstract]
  40. "Quantitative detection of single amino acid polymorphisms by targeted proteomics."J. Mol. Cell Biol. 3:309-315(2011) [PubMed] [Europe PMC] [Abstract]
  41. "A rare penetrant mutation in CFH confers high risk of age-related macular degeneration."Nat. Genet. 43:1232-1236(2011) [PubMed] [Europe PMC] [Abstract]