Factor H and disease: a complement regulator affects vital body functions.
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Factor H and disease: a complement regulator affects vital body functions. / Zipfel, P F; Hellwage, J; Friese, Manuel A.; Hegasy, G; Jokiranta, S T; Meri, S.
In: MOL IMMUNOL, Vol. 36, No. 4-5, 4-5, 1999, p. 241-248.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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T1 - Factor H and disease: a complement regulator affects vital body functions.
AU - Zipfel, P F
AU - Hellwage, J
AU - Friese, Manuel A.
AU - Hegasy, G
AU - Jokiranta, S T
AU - Meri, S
PY - 1999
Y1 - 1999
N2 - Factor H is a multidomain and multifunctional protein. As a complement regulator factor H determines the fate of newly formed C3b and controls formation and stability of C3 convertases both in the fluid phase and on cell surfaces. In addition, this plasma protein displays functions outside complement control as it has been suggested to act as an adhesion protein, to be a ligand for the cellular integrin receptor CR3 (CD11b/CD18) and to display chemotactic activity. Genetic and pathophysiological analyses describe a role for factor H in vital body functions. Depletion or the absence of factor H due to genetic reasons leads to unrestricted C3 consumption. A reduced amount of factor H in plasma or mutations within the factor H gene may lead to glomerulonephritis (type II MPGN) or hemolytic uremic syndrome (HUS). Certain pathogenic organisms have been shown to evade complement attack by binding factor H from the host. Such specific factor H binding components have been demonstrated on the surface of microbes, e.g., Streptococcus pyogenes and Neisseria gonorrhoeae. Here, we summarize the current knowledge how abnormalities in function of the central complement regulator factor H are associated with human diseases.
AB - Factor H is a multidomain and multifunctional protein. As a complement regulator factor H determines the fate of newly formed C3b and controls formation and stability of C3 convertases both in the fluid phase and on cell surfaces. In addition, this plasma protein displays functions outside complement control as it has been suggested to act as an adhesion protein, to be a ligand for the cellular integrin receptor CR3 (CD11b/CD18) and to display chemotactic activity. Genetic and pathophysiological analyses describe a role for factor H in vital body functions. Depletion or the absence of factor H due to genetic reasons leads to unrestricted C3 consumption. A reduced amount of factor H in plasma or mutations within the factor H gene may lead to glomerulonephritis (type II MPGN) or hemolytic uremic syndrome (HUS). Certain pathogenic organisms have been shown to evade complement attack by binding factor H from the host. Such specific factor H binding components have been demonstrated on the surface of microbes, e.g., Streptococcus pyogenes and Neisseria gonorrhoeae. Here, we summarize the current knowledge how abnormalities in function of the central complement regulator factor H are associated with human diseases.
M3 - SCORING: Zeitschriftenaufsatz
VL - 36
SP - 241
EP - 248
JO - MOL IMMUNOL
JF - MOL IMMUNOL
SN - 0161-5890
IS - 4-5
M1 - 4-5
ER -
