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).
Not exact matches
PRA is caused by the degeneration of the
photoreceptor cells,
rods and cones, which are needed
for dark and day light vision, respectively.
An international research team headed by Thomas Münch of the Institute
for Ophthalmic Research and Werner Reichardt Centre
for Integrative Neuroscience of the University of Tübingen found the contribution of
rod photoreceptors in mouse retinas to be much greater than previously assumed.
Rods and cones [
photoreceptors in the eye] could not account
for this differential regulation of melatonin production, so we postulated another type of
photoreceptor was responsible
for mediating such physiological responses.
The therapy employs a virus to insert a gene
for a common ion channel into normally blind cells of the retina that survive after the light - responsive
rod and cone
photoreceptor cells die as a result of diseases such as retinitis pigmentosa.
Retinal ganglion cell axons (arrowhead), RPE (arrow), and
rod photoreceptors (stained red
for XAP2) are donor derived.
(A) Rosettes (arrowheads) and pseudo outer nuclear layers (arrow) are detected in explants triple stained
for nuclei (blue; DAPI), cone (green; Calbindin), and
rod photoreceptors (red; XAP2).
The carboxyl - terminal domain is essential
for rhodopsin transport in
rod photoreceptors.
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).
Cone -
rod dystrophy first affects the cones in the retina, which are the
photoreceptors responsible
for detecting bright light or daylight.
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.
The
photoreceptors of dogs that carry this mutation develop normally, in contrast to those of dogs with XLPRA2, and remain morphologically and functionally normal until young adulthood, indicating the C - terminal of the RPGR protein is not essential
for functional and structural differentiation of
rods and cones.
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.
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.