Article
Low temperature completely rescues the function of two misfolded K ATP channel disease-mutants.
FEBS letters - 1 Aug 2005
Yang Ke, Fang Kun, Fromondi Laura, Chan Kim W
Abstract excerpt
The pancreatic ATP-sensitive potassium channels comprise two subunits: SUR1 and Kir6.2. Two SUR1 mutations, A116P and V187D, reduce channel activity causing persistent hyperinsulinemic hypoglycemia of infancy. We investigated whether these mutations cause temperature sensitive misfolding. We show that the processing defect of these mutants is temperature sensitive and these two mutations disrupt the association...
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