"Photoreceptor degeneration" refers to the process of progressive damage or loss of the cells in the eye responsible for detecting and conveying light signals to the brain.
Full definition
Light is known to induce nuclear c - Fos expression in the INL of the mouse retina, however this response is reduced in a mouse model of photoreceptor degeneration [34].
Thus, transplanted hNPCctx have the potential to release multiple growth factors, which may act synergistically to
slow photoreceptor degeneration [62], [63].
«We believe our results could eventually have a substantial impact on the development of treatments for retinal degenerative diseases, like retinitis pigmentosa and Leber's congenital amaurosis, by providing novel drug targets to
prevent photoreceptor degeneration,» concludes Dr. Cayouette.
Genetic defects
cause photoreceptor degeneration, which are majorly classified into rod - cone dystrophy called retinitis pigmentosa (rods initially degenerate, followed by cone degeneration), cone - rod dystrophy (cone degeneration, followed by rod degeneration), and Leber congenital amaurosis (LCA).
In this well - studied model of autosomal recessive retinitis pigmentosa and
secondary photoreceptor degeneration, a MERTK mutation in the RPE compromises their ability to phagocytose shed photoreceptor outer segments [35]--[37].
The retinal degeneration 6 (rd6) mutant is an autosomal recessive mouse model of retinal degeneration that was first detected by indirect ophthalmoscopy with white spots that were observable as early as 8 weeks of age and
exhibited photoreceptor degeneration.
Furthermore we analyse their functionality in vitro, and in vivo after transplantation of iPS - RPE into the dystrophic RCS rat: a model of retinal dystrophy where the primary defect, originating in RPE cells [26], leads to blindness as a consequence of rod and
cone photoreceptor degeneration [27]--[29].