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Progression of retinal disease linked to cell starvation
Date:12/7/2008

BOSTON, Mass. (Dec. 7, 2008) Rods and cones coexist peacefully in healthy retinas. Both types of cells occupy the same layer of tissue and send signals when they detect light, which is the first step in vision. The incurable eye disease Retinitis Pigmentosa, however, reveals a codependent relationship between the two that can be destructive. When flawed rods begin to die, otherwise normal cones follow them to the grave, leading to blindness. A new study might explain why.

Data published online in Nature Neuroscience Dec. 7 suggest the cones are starving to death. As rods disappear, the structure of the retina breaks down. This might disrupt the connections between the cones and their source of nutrients.

"This is the first study linking cone death in Retinitis Pigmentosa to a metabolic problem that suggests starvation," says senior author Constance Cepko, an HMS professor and investigator with Howard Hughes Medical Institute. "If we can find a way to supply nutrients to the cones, we might be able to preserve daylight vision in patients."

Active in bright light, cones allow us to perceive color and fine details. Conversely, rods allow us to see in dim light. The untrained eye cannot distinguish between the two types of cells, which grow side-by-side. Both rods and cones have a protrusion that has many membranous discs, resembling a stack of cookies. A cone stack is half the height of a rod stack. Stacks emanating from both types of cells get clustered together, like Oreos on a plate. The entire plate gets covered in "plastic," with the flexible plastic reaching down to touch each stack. In the eye, this plastic consists of a giant retinal pigment (RPE) cell, which supplies nutrients to the rods and cones on its plate.

With this structure in mind, researchers have proposed a variety of hypotheses to explain the loss of cones in patients with mutations in rod-specific genes. For example, some teams have suggested that r
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Contact: Alyssa Kneller
alyssa_kneller@hms.harvard.edu
617-432-0442
Harvard Medical School
Source:Eurekalert

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