The complement factor H-related proteins

Immunol Rev. 2016 Nov;274(1):191-201. doi: 10.1111/imr.12477.

Abstract

The role of the complement factor H-related (FHR) proteins in homeostasis, pathogen defense, and autoimmune disease has recently attracted considerable interest. We highlight the exciting research that has contributed to our understanding of the FHR protein family. Unlike factor H, a potent negative regulator of complement C3 activation, the FHR proteins appear to promote C3 activation. These data have important implications for understanding complement-mediated diseases because, depending on the context, the balance between the actions of factor H and the FHR proteins determines the degree of complement activation.

Keywords: complementinflammation; infection; kidney.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Autoimmunity
  • Blood Proteins / immunology
  • Blood Proteins / metabolism*
  • Complement Activation*
  • Complement C3 / metabolism*
  • Complement Factor H / immunology
  • Complement Factor H / metabolism*
  • Homeostasis
  • Humans
  • Immunity, Innate*

Substances

  • Blood Proteins
  • Complement C3
  • factor H-related protein 1
  • Complement Factor H