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Retinal Basal Laminar Deposits in Complement fH/fP Mouse Model of Dense Deposit Disease

Delu Song1, Imran Mohammed2, Rupak Bhuyan1

  • 1Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.

Investigative Ophthalmology & Visual Science
|July 20, 2018
PubMed

Insights

A new mouse model with mutations in complement factor H (fH) and factor properdin (fP) develops rapid-onset dense deposit disease (DDD) and retinal degeneration. This model offers insights into complement-mediated diseases like age-related macular degeneration.

Area of Science:

  • Ophthalmology
  • Nephrology
  • Immunology

Background:

  • Dense deposit disease (DDD) involves complement alternative pathway dysregulation, leading to kidney and eye pathology.
  • Complement factor H (fH) inhibits, while factor properdin (fP) promotes, alternative pathway activation.
  • Previous studies showed fH/fP double-mutant mice develop GBM deposits and nephropathy.

Purpose of the Study:

  • To characterize the ocular pathology in fH and fP double-mutant mice.
  • To establish a novel animal model for complement-mediated dense deposit disease.
  • To investigate the role of complement in basal laminar deposits (BLamD) relevant to AMD.

Main Methods:

  • Generated fH and fP double-mutant mice (fHm/m/fP-/-).
  • Performed fundus imaging, light/electron microscopy, and electroretinography (ERG).
  • Assessed complement levels and tested anti-C5 antibody treatment efficacy.

Main Results:

  • fHm/m/fP-/- mice exhibited early retinal hypopigmentation and basal laminar deposits (BLamD).
  • Photoreceptor degeneration and RPE vacuolization were observed.
  • ERG revealed diminished retinal function, which was improved by anti-C5 antibody treatment.

Conclusions:

  • This fH/fP double-mutant mouse is a valuable model for rapid-onset, complement-mediated DDD.
  • The model aids in studying BLamD pathology in age-related macular degeneration (AMD).
  • Targeting the complement cascade, specifically C5, shows potential therapeutic benefit.
Abstract

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