Cannon Lab

Ion Channelopathies of Skeletal Muscle

Studying Ion Channelopathies of Skeletal Muscle

The goal of our research program is to understand how mutations of ion channel genes expressed in skeletal muscle cause involuntary after-contraction (myotonia) and recurrent attacks of weakness (periodic paralysis). We leverage these insights to develop approaches grounded in science that reduce the frequency and severity of attacks through lifestyle changes, targeted application of drugs, or gene editing therapeutics.

Cannon Lab - Home Page Hero — The symptoms of myotonia and periodic paralysis occur in a broad spectrum of inherited muscle diseases, each of which is caused by ion channel gene mutations. Delayed relaxation of muscle force in myotonia is cause by anomalous bursts of electrical activity (bottom left); whereas the loss of force in periodic paralysis (bottom right) is cause by impaired excitability.

Focus of Our Lab

Our lab uses a variety of techniques to advance our understanding for the causes and opportunities for improving symptom management in periodic paralysis including:

Determining the effect of specific mutations on channel function.
Using computational models to explore the impact of altered channel behavior on muscle excitability.
Developing genetically engineered mouse models of periodic paralysis as a platform to discover how environmental stress (diet, exercise, temperature) triggers attacks of weakness and to test pre-clinical strategies for therapeutics and disease modification.

Learn more about our research program

Visit Cannon Lab Research Projects