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Discovery of source of glycogen 'manufacturing' errors sheds light on fatal disease
Date:3/1/2011

ists was the source of the phosphate. The answer, Dr. Roach and his colleagues report in Cell Metabolism, is found in the enzyme that cells normally use to build up the glycogen molecules. When there are extra glucose molecules in the body, such as after a meal, the enzyme, like a tiny molecular machine, hooks the glucose molecules onto the glycogen chain. In the process, phosphates that are attached to the glucose are discarded.

But once every 10,000 cycles or so, a phosphate molecule remains attached when the enzyme hooks the glucose molecule onto the glycogen chain. The job of laforin is to correct for those errors. Without the laforin, phosphates build up and the deadly disease is the result.

Dr. Roach and his colleagues note that animal experiments suggest that a treatment to counteract the laforin mutation could be compounds that would block the production of glycogen. Although a lack of glycogen could have side effects such as greater propensity to develop diabetes they might be acceptable in the face of such a deadly alternative, Dr. Roach said. However, such an approach is only a theoretical possibility at his point, he said.


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Contact: Eric Schoch
eschoch@iupui.edu
317-274-7722
Indiana University School of Medicine
Source:Eurekalert  

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Discovery of source of glycogen 'manufacturing' errors sheds light on fatal disease
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