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ALL EYES ON RETINAL DEGENERATION
February 16, 2010-Research by Johns Hopkins sensory biologists studying fruit flies, has revealed a critical step in fly vision. Humans with problems in this same step suffer retinal dystrophies, which manifest as visual defects ranging from mild visual impairments to complete blindness. The article, published Jan. 26 in Current Biology paves the way for using the fruit fly to screen for therapies to treat human retinal degeneration.
Retinal dystrophies result from inherited defects in nearly every step of the so-called “visual cycle,” a series of biochemical reactions known to occur in vertebrates, which recycles the molecule that enables light detection in eye cells. “Therapeutic approaches to tackle such retinal dystrophies are very limited,” says Craig Montell, Ph.D., a professor of biological chemistry and of neuroscience at the Johns Hopkins University School of Medicine. “So it’s useful to take advantage of simpler experimental model organisms, like fruit flies, to tease apart complex systems like vision, then translate that to use in vertebrates.”
This visual cycle previously was not thought to exist in invertebrate eyes. In fact, according to Montell, those who study fly vision long thought that as the molecules in the fly eye responsible for capturing photons of light can be regenerated by absorbing more light, they don’t need a visual cycle for the cells to reuse the molecule.
Curious about whether one particular enzyme in the fly eye — pigment-cell-enriched dehydrogenase (PDH) — plays a role in the fly’s ability to make the molecules that sense light, Montell and his research team generated flies carrying a mutation in the gene encoding PDH. They found the newly hatched flies lacking PDH to be totally normal in their ability to respond to light.
“It was a surprise. Initially the PDH looked dispensable as the visual responses were normal, but over time the pigment degraded,” says Montell. “This led us to ask the question: If PDH doesn’t make new light-sensing molecules, and flies can recycle them using light anyway, why are these flies losing their light-detecting molecules and consequently their sight?”
As it turns out, Montell and his team found PDH is required to help recycle the used light-capturing molecules in a previously unrecognized visual cycle in flies. Flies can recycle the molecules by absorbing light, but eventually the protein that holds the molecules in the cells needs to be replaced with new protein. When this happens the biochemical visual cycle is needed to regenerate the light sensing molecules. Over time, in pdh mutant flies, without a functional visual cycle, the used light-sensitive molecules were not regenerated causing cells in the retina to die, leading to vision loss.
To get an idea of how comparable the visual cycle is in flies and mammals, the team replaced the fly gene for PDH with a gene for a similar mammalian enzyme. These flies had normal electrical activity in cells of the retina in response to light, were able to maintain proper levels of light sensitive molecules, and had healthy retinas. This experiment showed the researchers that there are similarities in the visual cycles in mammals and flies.
“Flies are a good model in which to study and test new therapies for retinal degeneration,” says Montell. “This research opens the door to using flies as a way to look for drugs to reduce human retinal degeneration due to defects in the visual cycle.”
This study was funded by the National Eye Institute.
Authors of the text were Xiaoyue Wang, Tao Wang, Yuchen Jiao and Craig Montell from Johns Hopkins University and Johannes von Lintig from the Case Western Reserve University School of Medicine, Cleveland.
Source: http://www.hopkinsmedicine.org/Press_releases/2010/02_16_10.html
The Visual Eye
Visual Eye v2.0 -- an instructional flip-book Flash animation of the human eyeball.
Visual Eye covers 30 points of the human eyeball. To view this booklet, Adobe Flash player is required. The Flash player is free from Adobe, and may be downloaded from Adobe. While not required, Visual Eye is optimally viewed at a 1024x768 screen resolution. See changes file for version information.
Original artwork and text descriptions for Visual Eye were obtained through Wikipedia and Wikimedia Commons -- two of the very best places on the Web for accurate, open-sourced encyclopedic information and images. Visual Eye points to the aforementioned resources; notice the clickable links throughout the booklet. Please note however, that Wikipedia content is regularly refined by its expert contributors. As such, the description that we felt comfortable going with -- the one found in the Visual Eye booklet -- may not always exactly match the description found at the Wikipedia web site. Call it creative license, and the fact that we have our own accredited expert contributors on-hand. :)
Version 2 of Visual Eye weighs in at just under 5 megabytes. Once the booklet finishes loading, controls are straightforward. Specific points within the book may be reached from the Table Of Contents. To turn to the next page, click, or drag from any corner of the booklet.
Located above the booklet are major tabs to points TOC, 10, 20 and credits. With 30 total points covered across 65 pages, those particular pages seemed like ideal interim points to land on. (read: saves from a lot of page-turning.)
Launch Visual Eye (please be patient while Visual Eye loads -- it's almost 5M!)
Useful links
There are many sources of medical reference available on the Internet, and finding reputable sites can be challenging. Several excellent resources are listed below for your convenience.
- National Eye Institute -- http://www.nei.nih.gov
- American Optometric Association -- http://www.aoa.org
- Merck Manuals Online Medical Library -- http://www.merck.com/mmpe/sec09.html
- American Journal of Opthamology -- http://www.ajo.com
- Contact Lens Spectrum -- http://www.clspectrum.com/
- WebMD -- http://www.webmd.com
Frequently Asked Questions
1. How often should I have my eyes checked?
Dr. Leaks recommends that everyone have their eyes examined at least once a year and more often if you have conditions that warrant close monitoring such as diabetes, macular degeneration, glaucoma and cataract to name a few. In all such cases, the doctor will recommend an examination schedule for you, which may be different from someone else who has the same are similar disease.
2. Why go to the eye doctor if I can just go to the drug store and buy reading glasses?
Keep in mind that one does not always go to be eye doctor just to see better. Most of the time we should go to the doctor to make sure eyes are normal and healthy. In many cases, reading glasses are all that is needed, and if that is the case, drugstore glasses may be recommended. But we must be careful not to be tricked into thinking that our eyes a healthy just because we have good visual acuity.
3. How often should I undergo exams if I am a diabetic?
Every person is different and the exact eye exam schedule will be tailored to your particular case. For some people that means once a year. For others it may mean once every two months. Start with a simple office visit following the schedule outlined in FAQ question #1. Be sure to follow your doctor's advice on when you should be seen for follow-up visits.
4. If glaucoma or macular degeneration run in my family, should I take extra precautions?
There is a lot of evidence that many eye diseases, including macular degeneration and glaucoma, may run in families. For sure, if a close family member has one of these diseases, it increases your risk for having that disease. Therefore, extra precautions should be taken. If you've not been told that you have any signs of glaucoma on macular degeneration, then the doctor will probably recommend examinations once a year. If glaucoma or macular degeneration has been found during a regular examination, your doctor may recommend that you come in for more frequent eye examinations. In addition, the doctor may recommend that you take certain nutritional supplements that have been shown by studies and clinical trials to to help mitigate the effects of these diseases.
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Yes, during the months of August and September Dr Leaks provides free screenings for students grades K-12. A screening provides the doctor with the chance to catch a problem before a patient may even notice there is a problem. Should the patient need further treatment, such as corrective lenses; Dr Leaks will gladly provide a diagnostic and inform patients and guardians of appropriate corrective materials. Contact our office today to schedule your childs' appointment today.