Compstatin analog Cp40 inhibits complement dysregulation in vitro in C3 glomerulopathy

Highlights:
  • C3 glomerulopathy (C3G) defines a group of untreatable ultra-rare renal diseases caused by uncontrolled activation of the alternativecomplement pathway. Nearly half of patients progress to end stage renal failure within 10 years.
  • This study shows that Cp40 prevents complement-mediated lysis of sheep erythrocytes in sera from C3G patients, prevents complement dysregulation in the presence of patient-derived autoantibodies to the C3 and C5 convertases, and prevents complement dysregulation associated with disease-causing genetic mutations.
  • Conclusion: Cp40 may offer a novel and promising therapeutic option to C3G patients as a disease-specific, targeted therapy.
  • Author and source information

    Authors: Zhang Y, Shao D, Ricklin D, Hilkin BM, Nester CM, Lambris JD, Smith RJ.
    Reference: Immunobiology. 2015 Aug;220(8):993-8. doi: 10.1016/j.imbio.2015.04.001. Epub 2015 May 5.
    Source: www.ncbi.nlm.nih.gov