| The macula is located in the center of the retina | | | | the recessive gene - which means they do not |
| in the back of the eye. It is responsible for our | | | | know that they are carriers. The responsible |
| detailed and straight ahead vision - the vision that | | | | gene, ABCR, has recently been identified which will |
| is needed for seeing faces, driving and reading. | | | | be useful for future gene therapy. |
| Peripheral or side vision is usually not affected. It | | | | Unfortunately, there are few clinical trials for the |
| is not blindness. | | | | different forms of juvenile macular degeneration. |
| There are several forms of juvenile macular | | | | At this time (January 2009) there are none that |
| degeneration - all of them affect the macula, the | | | | are actively recruiting. |
| tiny spot in the back of the retina and all are | | | | This study sponsored by the National Eye institute |
| associated with a genetic mutation. | | | | was completed in early 2008 and we are still |
| Best Disease is also known as vitelliform macular | | | | waiting to hear the conclusions. |
| dystrophy. This disease is hereditary and is | | | | Effect of DHA Supplements on Macular Function in |
| passed on by a gene. The diagnosis is usually | | | | Patients With Stargardt Macular Dystrophy and |
| made during childhood or adolescence. There are | | | | Stargardt-Like Macular Dystrophy |
| several stages to this macular dystrophy. | | | | "As our North American diet is poor in DHA, we |
| 1) A yellow cyst forms under the retinal pigment | | | | hypothesize that a DHA dietary supplement might |
| epithelium (RPE) which is a layer under the macula | | | | improve macular function in individuals with the |
| - vision often remains normal for many years | | | | ELOVL4 mutation. Since the effect of DHA |
| 2) The cyst ruptures and the fluid and yellow | | | | supplementation may be non-specific, we propose |
| deposits flood the RPE | | | | to study a second cohort with Stargardt macular |
| 3) Fluid and deposits cause a degeneration of the | | | | dystrophy, which has a different genotype |
| RPE and the macula causing central vision loss | | | | involving a different metabolic pathway in the eye, |
| The patient still has peripheral vision. | | | | but presents with a similar phenotype. Two |
| Stargardt's disease is the most common form of | | | | cohorts of up to 10 subjects for analysis will be |
| juvenile macular degeneration. It was first | | | | recruited from patients with either Stargardt-like |
| discovered in 1901 by a German ophthalmologist, | | | | macular dystrophy or Stargardt macular |
| Karl Stargardt. This genetic form of macular | | | | dystrophy. |
| dystrophy is the result of both parents carrying | | | | |