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All eyes on retinal degeneration
Date:2/16/2010

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: I
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Contact: Audrey Huang
audrey@jhmi.edu
410-614-5105
Johns Hopkins Medical Institutions
Source:Eurekalert

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