Cone photoreceptor neuroprotection in inherited retinal degenerations
2012
High resolution and colour vision is derived from cone photoreceptors within the retina of the
eye. These highly specialised neuronal cells convert energy from incident light into changes
in cellular membrane potential. Action potentials are then relayed and processed within the
inner retina and the cerebral cortex. Loss of function in the cone photoreceptors is the direct
cause of visual loss for millions o f patients. Yet for many of the most common causes of
blindness cone photoreceptor dysfunction occurs late on in the disease and is secondary to
inherited and/or environmental influences that primarily affect other cell types. Cone
photoreceptors are then secondarily affected and it is once their function is lost that the patient
becomes visually disabled.
The aim of this thesis was to evaluate therapies targeting the molecular steps of cone
photoreceptor death rather than the underlying pathology. If effective at slowing cone
photoreceptor degeneration and preserving function, such therapies could be applicable to
large numbers of patients with a variety of underlying defects. There will however be
continued stimuli for cell death as the primary defect has not been corrected and so it is
important to determine the magnitude and duration of any treatment effect. The experiments
of this thesis were performed in an animal model of inherited retinal degeneration derived to
allow repeated in vivo assessments of cone photoreceptor function and survival. Gene
therapy and intra-ocular injections were used to evaluate proteins with contrasting modes of
action.
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