A skeletal muscle L-type Ca²⁺ channel with a mutation in the selectivity filter (Ca_V1.1 E1014K) conducts K^.

ABSTRACT: A glutamate-to-lysine substitution at position 1014 within the selectivity filter of the skeletal muscle L-type Ca²⁺ channel (Ca_V1.1) abolishes Ca²⁺ flux through the channel pore. Mice engineered to exclusively express the mutant channel display accelerated muscle fatigue, changes in muscle composition, and altered metabolism relative to wildtype littermates. By contrast, mice expressing another mutant Ca_V1.1 channel that is impermeable to Ca²⁺ (Ca_V1.1 N617D) have shown no detectable phenotypic differences from wildtype mice to date. The major biophysical difference between the Ca_V1.1 E1014K and Ca_V1.1 N617D mutants elucidated thus far is that the former channel conducts robust Na⁺ and Cs

SUBMITTER: Beqollari D 

PROVIDER: S-EPMC5836107 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature