Article
Malignant hyperthermia mutation sites in the Leu2442-Pro2477 (DP4) region of RyR1 (ryanodine receptor 1) are clustered in a structurally and functionally definable area.
The Biochemical journal - 1 Jan 2007
Bannister Mark L, Hamada Tomoyo, Murayama Takashi, Harvey Peta J, Casarotto Marco G, Dulhunty Angela F, Ikemoto Noriaki
Abstract excerpt
To explain the mechanism of pathogenesis of channel disorder in MH (malignant hyperthermia), we have proposed a model in which tight interactions between the N-terminal and central domains of RyR1 (ryanodine receptor 1) stabilize the closed state of the channel, but mutation in these domains weakens the interdomain interaction and destabilizes the channel. DP4 (domain peptide 4), a peptide corresponding to...
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