Causes and Risk Factors
Print Email

What causes Stargardt's disease?

In 1997, Foundation Fighting Blindness researchers isolated the gene for Stargardt's disease. The ABCR gene produces a protein involved in energy transport to and from photoreceptor cells in the retina. Mutations in the ABCR gene, which cause Stargardt's disease, produce a dysfunctional protein that cannot perform its transport function. As a result, photoreceptor cells degenerate and vision loss occurs.

Risk Factors

Stargardt's disease, an early-onset form of macular degeneration, is an inherited disease. The condition is programmed into your cells at conception. It is not caused by injury, infection or exposure to a toxic agent. Because Stargardt's disease is an inherited condition, there is nothing that can be done to reduce the risk of developing the disease.

However, recent findings in rodent models of Stargardt's disease find that unprotected, prolonged exposure to light can accelerate vision loss. Therefore, it is strongly recommended that patients with Stargardt's disease wear brimmed hats or visors and sunglasses when outdoors.

Stargardt's disease is an autosomal recessive disease. In autosomal recessive diseases, unaffected parents, who are carriers, have one gene with a disease-causing mutation paired with one normal gene. Each of their children then has a 25 percent chance (or one chance in four) of inheriting the two diseased genes (one from each parent) needed to cause the disorder. Carriers are unaffected because they have only one copy of the gene.

What can I do to reduce risk?

Unfortunately, there is no treatment that has been proven to improve the visual loss or to retard the progressing of the disease.