What is Stargardt Disease?





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Published on Apr 15, 2014

0:05 Narrator: (Animation) In Stargardt Disease, a defective protein in photoreceptors results in the accumulation of lipofuscin in retinal pigment epithelial or RPE cells as photoreceptors are shed daily. Lipofuscin is composed of material that is very toxic to RPE cells. The RPE cells become engorged with this toxic material and over time begin to shrink and die. Since RPE cells are important for the survival of photoreceptors, when RPE cells are lost, so too are the photoreceptors that lie directly above them, and this leads to blindness.

0:41 Steven Nusinowitz: If you were patient with Stargardt's disease and you were looking at my face, if we were early in the disease course my face might appear quite blurry and my features may not be recognizable. Whereas the peripheral parts of the retina would be perfectly okay. In later course of the disease, where you are actually losing a lot of cells in the macular region, my face would be entirely black. Again, peripheral parts of the retina would be fine. In order to see my face patients would typically look slightly askew so that my face falls on parts of the retina that are not affected by the Stargardt's gene.

Steven Nusinowitz, PhD
Jules Stein Eye Institute





American Macular Degeneration Foundation
P.O. Box 515
Northampton, Massachusetts 01061-0515
1.888.MACULAR (1.888.622.8527)

Welcome to the American Macular Degeneration Foundation where you can learn about this disease, find valuable resources and help conquer macular degeneration.

Founded on June 1, 1996

Few people are aware that macular degeneration is an incurable eye disease and that it is the leading cause of vision loss for those aged 55 and older in the United States, affecting more than 10 million Americans.

Macular degeneration is caused by the deterioration of the central portion of the retina, the inside back layer of the eye that records the images we see and sends them via the optic nerve from the eye to the brain. The retina's central portion, known as the macula, is responsible for focusing central vision in the eye, and it controls our ability to read, drive a car, recognize faces or colors, and see objects in fine detail.

As people age, their chances for developing eye diseases increase dramatically. Unfortunately, the specific factors that cause macular degeneration are not conclusively known and research into this little-understood disease is limited by insufficient funding.

The American Macular Degeneration Foundation is committed to the prevention and cure of macular degeneration and offers hope and support to those afflicted and their families. The Foundation is a voice in establishing the national research agenda for macular degeneration through promoting an alliance among the scientific community, government, and victims of the disease and their families to ensure the prevention and cure of the disease.

The AMDF is a 501(c)(3) non-profit, publicly supported organization.

There are two basic types of macular degeneration: "dry" and "wet." Approximately 85% to 90% of the cases of macular degeneration are the "dry" (atrophic) type. Approximately 10-15% of the cases of macular degeneration are the "wet" (exudative) type.

The AMDF Web site offers many tools to help you better understand and cope with macular degeneration. We encourage you to visit our website, and contact us.

Mission: The American Macular Degeneration Foundation works for the prevention, treatment, and cure of macular degeneration through raising funds, educating the public and supporting scientific research.


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