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CFHR4  -  complement factor H-related 4

Homo sapiens

Synonyms: CFHL4, Complement factor H-related protein 4, FHR-4, FHR4
 
 
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Disease relevance of CFHR4

  • Recombinant FHR-4 protein was expressed in the baculovirus system and has an apparent molecular mass of 42 kDa [1].
 

High impact information on CFHR4

  • A protein consisting of SCRs 19 and 20 did not bind heparin, whereas SCRs 18-20 did, indicating that, although SCR 20 contains a heparin binding site, at least two nonspecific adjacent SCRs are required. fH-related protein-3 (FHR-3) possesses an SCR homologous to SCR 7 of fH and bound heparin, whereas FHR-4, which lacks such an SCR, did not [2].
  • In parallel we identified the cDNA encoding a new complement factor H-related protein, termed FHR-4 [1].
  • The human factor H-related protein 4 (FHR-4). A novel short consensus repeat-containing protein is associated with human triglyceride-rich lipoproteins [1].
  • The sequences of the new apoprotein overlapped with that of the FHR-4 protein [1].
  • Genetic analysis shows that the human FHR-4 gene is composed of 10 coding exons, and two distinct mRNA transcripts are derived from this gene by alternative splicing [3].
 

Biological context of CFHR4

  • Functional analyses show dose-dependent binding of recombinant FHR-4 to C3b, thus demonstrating a functional relatedness between FHR-4, factor H and FHL-1 and other complement regulators of the RCA gene cluster [4].
 

Associations of CFHR4 with chemical compounds

  • The interaction of FHR-3, but not of FHR4, with opsonised pneumococci was inhibited by heparin [5].
 

Other interactions of CFHR4

  • The function of the factor H-related proteins (FHR-1 to FHR-4) is currently under investigation [6].
  • In plasma FHR-4 occurs exclusively in a dimeric form, that most likely represents a homodimer consisting of two identical FHR-4 monomers [4].

References

  1. The human factor H-related protein 4 (FHR-4). A novel short consensus repeat-containing protein is associated with human triglyceride-rich lipoproteins. Skerka, C., Hellwage, J., Weber, W., Tilkorn, A., Buck, F., Marti, T., Kampen, E., Beisiegel, U., Zipfel, P.F. J. Biol. Chem. (1997) [Pubmed]
  2. Identification of the second heparin-binding domain in human complement factor H. Blackmore, T.K., Hellwage, J., Sadlon, T.A., Higgs, N., Zipfel, P.F., Ward, H.M., Gordon, D.L. J. Immunol. (1998) [Pubmed]
  3. FHR-4A: a new factor H-related protein is encoded by the human FHR-4 gene. Józsi, M., Richter, H., Löschmann, I., Skerka, C., Buck, F., Beisiegel, U., Erdei, A., Zipfel, P.F. Eur. J. Hum. Genet. (2005) [Pubmed]
  4. Biochemical and functional characterization of the factor-H-related protein 4 (FHR-4). Hellwage, J., Skerka, C., Zipfel, P.F. Immunopharmacology (1997) [Pubmed]
  5. Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and differential regulation by heparin. Hellwage, J., Jokiranta, T.S., Koistinen, V., Vaarala, O., Meri, S., Zipfel, P.F. FEBS Lett. (1999) [Pubmed]
  6. The factor H protein family. Zipfel, P.F., Jokiranta, T.S., Hellwage, J., Koistinen, V., Meri, S. Immunopharmacology (1999) [Pubmed]
 
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