Hemolytic Tests Exploring Factor H Functional Activities

Methods Mol Biol. 2021:2227:69-81. doi: 10.1007/978-1-0716-1016-9_7.

Abstract

Impairment of the complement regulatory protein Factor H (FH) is implicated in the physiopathological mechanisms of different diseases like atypical hemolytic and uremic syndrome and C3 glomerulopathies. It may be due to genetic abnormalities or acquired with the development of autoantibodies. FH has several ligands; therefore, the exploration of its functions requires to perform different tests. Among them, two hemolytic tests are very useful because they give specific and complementary information about FH functions. The first one is dedicated to explore the FH capacity to dissociate the alternative pathway C3 convertase, whereas the second one is designed to explore the capacity of FH to bind cell surfaces and to protect them from complement attack. This chapter describes the procedures to perform these two hemolytic tests, exploring in a complementary way the FH functionality.

Keywords: Autoantibody; Cell protection; Decay of C3 convertase; Factor H; Genetic abnormality; Hemolytic tests.

MeSH terms

  • Animals
  • Atypical Hemolytic Uremic Syndrome / blood
  • Atypical Hemolytic Uremic Syndrome / diagnosis
  • Atypical Hemolytic Uremic Syndrome / immunology
  • Complement C3b / analysis
  • Complement C3b / metabolism
  • Complement Factor H / analysis*
  • Complement Factor H / physiology*
  • Complement Hemolytic Activity Assay / methods*
  • Cytapheresis / methods
  • Erythrocytes / cytology
  • Erythrocytes / metabolism
  • Humans
  • Kidney Diseases / blood
  • Kidney Diseases / diagnosis
  • Kidney Diseases / immunology
  • Rats
  • Sheep

Substances

  • Complement C3b
  • Complement Factor H