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Elevated factor H-related protein 1 and factor H pathogenic variants decrease complement regulation in IgA

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Elevated FH-related protein 1 (FHR-1) levels and ratios contribute to IgA nephropathy (IgAN) progression by interfering with factor H (FH) complement regulation. Kidney function decline exacerbates this effect in IgAN and ADPKD.

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Area of Science:

  • Nephrology
  • Immunology
  • Complement System Biology

Background:

  • IgA nephropathy (IgAN) is a primary cause of chronic kidney disease.
  • Mesangial deposition of galactose-deficient IgA1 immune complexes characterizes IgAN.
  • Complement system activation is implicated in IgAN pathogenesis.

Purpose of the Study:

  • To investigate the correlation between clinical progression and levels of factor H (FH) and FH-related protein 1 (FHR-1) in IgAN and ADPKD patients.
  • To explore the role of FHR-1/FH ratio in the context of renal function impairment.

Main Methods:

  • Analysis of well-characterized patient cohorts: 112 IgAN, 46 ADPKD, and 76 controls.
  • Quantification of FH and FHR-1 levels and their ratios.
  • Examination of genetic variants in CFH and CFI.

Main Results:

  • Patients with IgAN and ADPKD exhibited elevated FHR-1 levels and FHR-1/FH ratios compared to controls.
  • Higher FHR-1 levels and ratios were observed in IgAN patients with disease progression and in ADPKD patients with chronic kidney disease.
  • Homozygous deletion of CFHR3/CFHR1 (ΔCFHR3-CFHR1) protected against IgAN, but not ADPKD.

Conclusions:

  • Decreased FH activity in IgAN may result from increased FHR-1/FH competition or pathogenic CFH variants.
  • Elevated FHR-1 levels, potentially exacerbated by declining renal function, can worsen IgAN pathogenesis.
  • FHR-1 plays a role in IgAN by competing with FH for complement regulation.