Retinitis pigmentosa refers to a family of inherited photoreceptor degenerations resulting in blindness. During and after photoreceptor loss, neurons of the inner retina are known to undergo plastic changes. Here, we have investigated in detail whether ganglion cells are altered at late stages of degeneration, well after the total loss of photoreceptors. We used mice, rd1-Thy1, that carry a mutation in the β-subunit of phosphodiesterase 6 and a fluorescent protein that labels a subset of ganglion cells and B6-Thy1 control mice. Retinal wholemounts from mice aged 3-11 months were processed for immunohistochemistry and analyzed. Ganglion cells were classified based on soma area, dendritic field size, and branching of dendrites. The dendritic fields of some ganglion cells were further analyzed for their length, area and quantity of branching points. There was a decrease in size and level of branching of A2, B1, and D type ganglion cells in the degenerated retina at 11 months of age. In contrast, C1 ganglion cells remained unchanged. In addition, there was a shift in the proportion of ganglion cells ramifying in the different layers of the inner plexiform layer. Careful analysis of the dendrites of ganglion cells revealed some projecting to new, more distal regions of the inner plexiform layer. We propose that these changes in ganglion cell morphology could impact the function of individual cells as well as the retinal circuitry in the degenerated retina.
Vision-impaired individuals often use a long white cane to assist them with gathering information about their surroundings. However, these aids are generally not used to detect obstacles above knee height. The purpose of this study is to determine whether a low-cost, custom-built electronic device clipped onto a traditional cane can provide adequate vibratory warning to the user of obstacles above knee height. Sixteen normally sighted blindfolded individuals participated in two mobility courses which they navigated using a normal white cane and a white cane with the electronic device attached. Of the 16 participants, 10 hit fewer obstacles, and 12 covered less ground with the cane when the electronic device was attached. Ten participants found navigating with the electronic device easier than just the white cane alone. However, the time taken on the mobility courses, the number of collisions with obstacles, and the area covered by participants using the electronic device were not significantly different (p > 0.05). A larger sample size is required to determine if the trends found have real significance. It is anticipated that additional information provided by this electronic device about the surroundings would allow users to move more confidently within their environment.
These findings have helped to accurately trace the periods of photoreceptor degeneration in this model of RP and show that correct light-regulated inner retinal activation is maintained until the time of cone degeneration.
Retinal prostheses aim to restore vision to patients who are blind from photoreceptor diseases such as Retinitis Pigmentosa (RP). All implants target the neural cells in the inner retina, the retinal ganglion cells (RGCs). Our research focuses on further understanding the disease process of RP during mid to late stages when total loss of photoreceptors has occurred and significant remodeling of inner retinal neurons has taken place. We have used a novel transgenic mouse, Rd1-FTL, to observe different degenerative stages of RP. Notably, in the aged retina we have evidence that there was gross inner retinal remodeling as well as glial dysfunction that occurred in confined regions in the central retina that worsened overtime. Consequently, the timing of implantation and location of the prosthesis both need to account for the state of the retina at different stages in the disease process.
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