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Cell Death Pathways in Mutant Rhodopsin Rat Models Identifies Genotype-Specific Targets Controlling Retinal

Ishaq A Viringipurampeer1, Cheryl Y Gregory-Evans1, Andrew L Metcalfe1

  • 1Department of Ophthalmology and Visual Sciences, University of British Columbia, 2550 Willow Street, Vancouver, BC, V5Z 3N9, Canada.

Molecular Neurobiology
|June 19, 2018
PubMed

Insights

Retinitis pigmentosa (RP) treatments require genotype-specific strategies. Targeting cell death pathways with drug combinations shows promise for preserving vision in RP patients, particularly for cone photoreceptor survival.

Area of Science:

  • Ophthalmology
  • Neuroscience
  • Genetics

Background:

  • Retinitis pigmentosa (RP) is an inherited retinal disease causing progressive vision loss due to photoreceptor cell death.
  • Current treatments for RP are limited, highlighting the need for novel therapeutic strategies.
  • Understanding the molecular mechanisms driving photoreceptor cell death is crucial for developing effective interventions.

Purpose of the Study:

  • To identify the molecular drivers of rod and cone cell death in two distinct RP rat models (S334ter and P23H rhodopsin).
  • To evaluate the efficacy of single and combination drug therapies targeting cell death pathways.
  • To determine if genotype-specific treatments are necessary for RP.

Main Methods:

  • Utilized transgenic S334ter and P23H rhodopsin rat models of retinitis pigmentosa.
  • Investigated cell death mechanisms including caspase-dependent apoptosis and RIP3-dependent necroptosis.
  • Administered specific cell death inhibitors and combination therapies to assess retinal function and cell survival.

Main Results:

  • In the S334ter model, rod death was caspase-dependent, while cone death involved necroptosis.
  • In the P23H model, rod death was necroptotic, and cone loss resulted from inflammasome activation.
  • Combination therapy improved outcomes in the P23H model but not the S334ter model, indicating genotype-specific responses.

Conclusions:

  • Different RP genotypes utilize distinct cell death pathways.
  • Genotype-specific combination therapies are essential for effectively treating RP and preserving cone photoreceptor survival.
  • Tailored therapeutic approaches are critical for managing inherited retinal degenerations.
Keywords:
ApoptosisNecroptosisNeuroprotectionPersonalized medicineRetinal degenerationRhodopsin

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