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Attacks of King George III's madness linked to key metabolism molecule

p>Through a series of experiments with normal mice and those engineered to lack the PGC-1 alpha gene, the researchers said they have provided "a clear-cut mechanism" linking fasting to an increase in PGC-1 alpha, and, in turn, overactivity of the ALAS-1 gene. The therapeutic effect of glucose and high-carbohydrate diets on porphyria, they add, occurs because glucose causes the pancreas to make more insulin, which results in suppression of the PGC-1 alpha gene.

The findings suggest that patients with porphyria should avoid any drugs or foods that turn on PGC-1 alpha activity in the liver, the researchers say. There could be implications for treatment as well: high-carbohydrate diets aren't a satisfactory therapy for affected patients as it make them gain, and fasting in order to lose the weight risks provoking attacks. Hopefully, the researchers say, it might be possible to develop more specific treatments now that the mechanism underlying the symptoms of porphyria is better understood.


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Source:Dana-Farber Cancer Institute


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