The retinal pigment epithelium (RPE) is a monolayer of cells, residing at the back of the eye between Bruch's membrane and the retina, which is essential
for photoreceptor function and survival.
Not exact matches
The six previously known fly rhodopsins account
for the full
function of
photoreceptor cells in the fly's eyes, so although the fruit fly genome contained the sequence of a seventh rhodopsin, the role of Rh7 was unclear.
The receptor also regulates DHA retention and conservation in cells in the eye and is necessary
for photoreceptor cell
function.
Mice lacking the cryptochrome 2 blue - light
photoreceptor gene (mCry2) were tested
for circadian clock - related
functions.
The findings from our proposed studies will further define how the RPE supports the
photoreceptor functions via metabolic coupling, and hopefully identify novel druggable targets
for preventing RPE and retinal degeneration during AMD.
While preservation of the ONL is evidence
for a neuroprotective role
for hNPCctx and GDNF within the retina, maintenance of visual
function at the level observed in this study suggests at least partial retention of
photoreceptor structure necessary
for visual processing, particularly that associated with cones.
One can imagine that loss of
function of any of the gene products along this complex pathway could lead to severe consequences
for the survival of the
photoreceptor (PR) cells in the eye.
«
For age - related macular degeneration, the best timing might be when the
photoreceptors have lost
function but haven't yet died off,» she says.
This Clinical Statement provides recommendations
for patients with inherited retinal degenerations, which comprise a wide range of genetically and phenotypically heterogeneous diseases associated with progressive loss of
photoreceptor function and visual loss.
As a monolayer of cells critical to
photoreceptor function and survival, the RPE is an ideally accessible target
for cellular therapy.
Although known to have neuronal cell
functions STK38L has not previously been associated with abnormal
photoreceptor function; being associated with such a disease in dogs establishes this gene as a potential candidate
for similar diseases in other species, including man.
A subsequent electroretinography study identified an initial reduction of the cone
photoreceptor function which led to the condition being re-classified as a cone - rod dystrophy (CRD), rather than a rod - led PRA, and the disease was termed CORD1
for cone - rod degeneration 1 [36].
Intriguingly, an identical homozygous mutation was identified in a human patient with recessive retinitis pigmentosa, the human equivalent of PRA, and established the novel retinal gene, PRCD, as an important gene
for the maintenance of rod
photoreceptor structure and
function across species.
Whereas the genes implicated in early - onset diseases are those necessary
for the correct development of
photoreceptors the genes associated with later - onset forms of disease are those necessary
for the long - term maintenance and
function of these cells.
In general, PRAs are characterized by initial loss of rod
photoreceptor function followed by that of the cones and
for this reason night blindness is the first significant clinical sign
for most dogs affected with PRA.