Progressive retinal atrophy (PRA) and cone - rod dystrophy (CRD) are collective terms for two broad forms of progressive, bilateral degenerative diseases that affect
the retinal photoreceptor cells.
The RPE is a single layer of cells lining the back of the retina that is vital to the functioning of
the retinal photoreceptor cells, and thus vision itself.
A new gene therapy treatment has restored some sight in a handful of blind patients suffering from Leber's congenital amaurosis, a syndrome in which, because of a broken or missing gene called RPE65,
retinal photoreceptor cells malfunction and eventually die.
Not exact matches
Similar patches and treatments have already been tested against the «wet» form — in which blood vessels invade and destroy the
retinal pigment epithelial
cells that nourish and support the
photoreceptor cells that capture light.
The
retinal pigment epithelium (RPE) is a single layer of
cells that accomplishes multiple functions, such as providing survival molecules that prevent
photoreceptors from dying.
It first targets a protective lining called the
retinal pigment epithelium (RPE), which shuttles nutrients to the
photoreceptor cells and is vital for their survival.
These findings have suggested the development stages at which to transplant
cells — for instance,
photoreceptor cells need to be relatively more mature than stem
cells, according to Thomas Reh, who studies
retinal development at the University of Washington.
«This is important because the death of
photoreceptor cells is known to cause
retinal degenerative diseases in humans that lead to blindness.
Isacoff, Flannery and UC Berkeley colleagues have developed several optogenetic techniques for restoring light - sensitivity to surviving
retinal cells other than the
photoreceptors.
In normal mice with working
photoreceptors (PR driven), stimulating the retina produces a variety of responses in
retinal ganglion
cells, the output of the eye.
Bypassing damaged
retinal cells The light - sensitive
photoreceptors made by the rod and cone
cells in the retina also belong to the GPCR class.
Recent evidence suggests that the entraining
photoreceptors are
retinal ganglion
cells (RGCs) that project to the SCN.
When these
photoreceptors detect light, they send a signal to specialized neurons in the retina called
retinal ganglion
cells, or RGCs, which then transmit visual information to the brain by firing electrical pulses along the optic nerve.
These vessels supply oxygen and nutrients to the
retinal pigment epithelium and
photoreceptor cells and carry away waste products.
The researchers injured the mice retinas with a toxin that causes
cell death in
retinal ganglion
cells and interneurons, another type of
retinal cell whose job it is to transmit signals from
photoreceptors to the brain.
In wild - type,
retinal ganglion
cell layer (GCL), inner nuclear layer (INL), inner plexiform layer (IPL), and nuclear layers of rod and cone
photoreceptors are distinct, and rod outer segment (OS) is observed at the outer-most layer of the retina.
«Because degradation of
photoreceptors is believed to be a major factor in
retinal diseases, such as retinitis pigmentosa and Leber's congenital amaurosis, this finding, that horizontal
cells are necessary for the normal survival of
photoreceptor cells, is novel and significant,» says Mu.
«Many
retinal diseases are manifested by the degeneration of
photoreceptor cells.»
However, both Goldberg and Albini agreed there is no evidence that this kind of therapy could have treated the patients» sight problems even if carried out correctly and only sparse evidence that adipose
cells can differentiate into
retinal pigment epithelium or
photoreceptor cells, which play a critical role in macular degeneration.
Retinal ganglion
cell axons (arrowhead), RPE (arrow), and rod
photoreceptors (stained red for XAP2) are donor derived.
The second step will be to optimize culture conditions for the commitment of the iPS / hES
cell - derived
retinal precursors towards the
photoreceptor lineage.
They play a pivotal role in regulating synaptic transmission, modulating excitotoxicity responsible for much of the neuronal damage caused by hypoxic insult in the brain [37], and are expressed in
retinal photoreceptors, horizontal
cells, and bipolar
cells as well as the amacrine and ganglion
cells of the inner retina [38 — 41].
In Figure 5O, the number of rod
photoreceptor, inner nuclear layer, and
retinal ganglion
cells were determined by counting the nuclei of
cells expressing XAP2, Calretinin, or in the RGC layer, respectively.
Mouse models of induced pluripotent stem (IPS)
cells have demonstrated the feasibility of regenerating
photoreceptor cells and / or
retinal tissue.
When transplanted to the subretinal space of mice lacking functional
photoreceptors, human embryonic stem
cells directed toward a
retinal lineage integrate into the outer nuclear layer, express
photoreceptor markers, and restore a light response as determined by the electroretinogram (ERG)[5].
Molecular markers for
retinal ganglion, amacrine, bipolar, horizontal, Müller glia, and rod and cone
photoreceptor cells (Table S3) identified these
cell types (Figures 5B, 5D — 5N, and S2, S3, S4, S5, S6).
It results from the death of
cells critical for vision:
retinal pigment epithelial
cells (RPE) and
photoreceptor cells.
The
retinal pigment epithelium (RPE) is a layer of
cells next to the retina that are metabolically coupled to the retina's
photoreceptor neurons.
0 Research ArticleRETINAL DISEASES Human ESC — derived
retinal epithelial
cell sheets potentiate rescue of
photoreceptor cell loss in rats with
retinal degeneration Karim Ben M'Barek 1,2,3, Walter Habeler1, 2,3, Alexandra Plancheron1, 2,3,
In
retinal diseases such as age - related macular degeneration, for example,
photoreceptor cells that absorb light signals are damaged or dead.
His research is focused on
retinal regeneration by reprogramming human fibroblasts either into induced pluripotent stem
cells or directly into
photoreceptors.
In the absence of myosin VI, the amplitudes of the a - and b - waves of the electroretinogram were reduced, although there was not
photoreceptor cell loss and
retinal anatomy appeared normal.
Our results indicate that myosin VI is required in
photoreceptor cells for normal
retinal electrophysiology.
With age, our eyes accumulate waste in
retinal pigment epithelium (RPE), which supports the life and function of
photoreceptors (light sensitive
cells in the eye); in advanced stages, RPE and
photoreceptors die.
Behind the
photoreceptors is another layer of
cells called
retinal pigment epithelium (RPE), which support the rods and cones by delivering nutrients from the bloodstream and removing waste that the rods and cones generate.
These
cells can develop into any type of
retinal cell, including RPE
cells or
photoreceptors.
The treatment could help people who have a fault in a gene called RPE65, which causes problems in the
retinal pigment epithelium (RPE), a thin layer of
cells that support and nourish
photoreceptors.
In the past, neural stem and progenitor
cells from various sources were introduced into eyes with the thought that they might differentiate and replace
photoreceptors lost in
retinal disease [13]--[19].
Both unmodified and genetically modified groups were found to have
cells that migrated and survived in two distinct locations: (i) as a separate, nearly continuous, subretinal layer lying between the host RPE and
photoreceptors, and (ii) as individual
cells distributed throughout the neurosensory retina, especially within the inner
retinal layers (Figure 5A).
These
retinal cells are the type that are killed off in macular degeneration, eventually leading to the death of
photoreceptors, and the gradual loss of central vision.
These
cells protect and nourish the retina, remove waste products, prevent new blood vessel growth into the
retinal layer, and absorb light not absorbed by the
photoreceptor cells; these actions prevent the scattering of the light and enhance clarity of vision.
Fresh porcine
retinal tissue was dissected, orientated on a 0.45 mm filter with the
photoreceptor cell surface uppermost and placed on top of the iPS - RPE monolayer so that outer segments were adjacent to the RPE.
As these factors can exert protective effects on
retinal neurons [52]--[56] and neurons in other neurodegenerative disease models [49], [57], [58], it seems likely that these substances may contribute to the latent
photoreceptor cell survival we observe in the dystrophic retina.
Our objective is to determine whether this mutation is involved in AMD by (1) understanding the functional consequences of this genomic variation on expression of COL8A1 and / or its neighbor genes, and (2) investigating
photoreceptors and RPE
cell survival,
retinal and choroidal angiogenesis, structure integrity of the choroid / Bruch's membrane, and the quality of the vision, in organisms with the COL8A1 mutation.
These data suggest that human iPS - RPE
cells are able to contribute to host
photoreceptor cell integrity by removing
retinal debris at this time - point.
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.
Melatonin production is suppressed when the
retinal ganglion
cells of the eye detect sunlight and produce the
photoreceptor melanopsin.
However, the evidence from the past decade or so has revealed a third class of
photoreceptor within the eye; intrinsically photosensitive
retinal ganglion
cells (ipRGCs), which express their own distinct opsin — melanopsin [6,7].
To further examine the morphology of
cells and the localization of protein expression within the retina, immunohistochemical staining of both paraffin and OCT
retinal sections was performed with the following antibodies (Table S1): human cone arrestin (for cone
photoreceptors), rhodopsin (for rod
photoreceptors), RPE65 (for the
retinal pigment epithelium, RPE), glial fibrillary acidic protein (GFAP, for astrocytes and Müller
cells), glutamine synthetase (for Müller
cells) and G0alpha (for ON bipolar
cells).
Layers of the retina: RPE,
retinal pigment epithelium; PR,
photoreceptors; ONL, outer nuclear layer; OPL, outer plexiform layer; INL, inner nuclear layer; IPL, inner plexiform layer; GCL, ganglion
cell layer; NFL, nerve fiber layer.