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Atlanta (UPI) Jun 7, 2010 U.S. scientists say they've discovered commonalities between flies and humans that make flies a valuable new model for the metabolic disease galactosemia. The researchers said galactosemia results from an inherited genetic defect that prevents the proper metabolism of galactose, a sugar commonly found in dairy products. People with galactosemia have genetic mutations that decrease their levels of the key enzymes GALT and GALE, which are responsible for the metabolism of a common form of dietary sugar. Exposure of affected people to galactose can damage most of their organ systems and can be fatal. The ability to study the disease has been limited by a lack of animal models. But recent studies suggest similarities between humans and flies may provide scientists with useful clues. Scientists at Emory University developed fruit flies (Drosophila melanogaster) that carry genetic changes similar to those found in patients with galactosemia. Like patients with classic galactosemia, flies that are missing GALT survive if they are raised on food that does not contain galactose. Flies with impaired GALE function also succumb in greater numbers when exposed to galactose during development, similar to patients with defects in the same area of their metabolic pathway. The researchers said those models can help science understand how changes in sugar metabolism lead to disease and open the door to novel drug discoveries by serving as a testing ground for candidate therapeutics. The study appears in the journal Disease Models & Mechanisms.
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