Integrated effects of altered action potentials and calcium release on skeletal muscle force generation in transgenic Huntington’s disease mice

Huntington’s disease (HD) is a movement disorder commonly recognized as being neurodegenerative. An increasing number of studies also show primary HD dysfunction in multiple tissues, consistent with the widespread expression of the mutated huntingtin gene. Studies of HD skeletal muscle have revealed membrane hyperexcitability and prolonged action potentials due to Cl– and K+ channel dysfunction as well as decreased Ca2+ release from the sarcoplasmic reticulum (SR) due to ryanodine receptor dysfu