After years of searching hard for the so -
called photoreceptor cells that relay that light signal to the clock in mammals, circadian biologists had tantalizing clues but no answer.
Nevertheless, the outcome may pave the way for transplants of stem cell — derived eye cells
called photoreceptors, which could dramatically improve vision in people with eye disease if all goes according to plan.
Not exact matches
The retina contains small, specialized neurons
called bipolar cells that transmit information from light - sensitive
photoreceptor cells to ganglion neurons, which send information to the brain for interpretation as images.
In the new study, the team focused on neurons
called amacrine cells and horizontal cells, which have a known role in «preprocessing» — or adjusting — electrical signals transmitted to the brain from the
photoreceptors after they have been stimulated by light photons.
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.
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.
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.
A healthy retina usually features light - sensitive cells —
photoreceptors —
called cones and rods.
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).
The mutant —
called gold rush (gosh)-- presents a genetic mutation in the Aipl1b DNA sequence, losing Aipl1 activity in cone
photoreceptors.
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Called achromatopsia, the hereditary disease affects about one in 30,000 people in the U.S. Afflicted individuals can only see with 5 percent of their
photoreceptors and thus have a very low level of vision.
The mutant -
called gold rush (gosh)- presents a genetic mutation in the Aipl1b DNA sequence, losing Aipl1 activity in cone
photoreceptors.
With this approach the authors report a number of unique RP mutations, a previously undescribed disease gene
called NEK2 that involves the retinal
photoreceptors, and structural DNA rearrangements originating in introns.
The team transplanted stem cell - derived
photoreceptors into another strain of mouse,
called CRX null, which is congenitally blind.
I became an eye researcher completely by accident while trying to unravel the role of a protein
called Ankyrin in forming biological important membrane domains in cells, in this case, the
photoreceptor.
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.
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.
The macula is densely packed with
photoreceptor cells
called rods and cones that react to light and send electrical nerve impulses to the optic nerve and into the brain.