Mark A. Tarnopolsky presents at AMDA/IPA Conference

Mark A. Tarnopolsky presents at AMDA/IPA Conference

McMaster University, Hamilton, Ontario, Canada.

Posted On: 08/05/2020 Recently Posted!

Mark Tarnopolsky will be following his excellent and comprehensive paper on Nutrition and exercise in Pompe disease with a presentation on the topic at the San Antonio Pompe conference. Hopefully he'll have updates on many of his observations, including those on the benefits of a ketogenic diet.

"A ketogenic diet is usually >60% fat with very low carbohydrate (<50 g/d). The possible theories suggested for improving PD are that ketones (B-OH-butyrate and acetoacetate) can enhance muscle mass in MD, lower inflammation, activate chaperone-mediated autophagy, and that a very low carbohydrate intake is a form of substrate reduction therapy (SRT). The potential benefit of SRT was shown in the murine Pompe model with genetic knock-down of glycogen synthase leading to improved function. Practical issues with the ketogenic diet are issues of long-term compliance and care to ensure adequate micronutrient intake. Another concern is the potential to increase neutral lipids in the liver and lead to non-alcoholic fatty liver disease (NAFLD) and hepatic insulin resistance. Clearly, with the anecdotal evidence of “benefit” it is important that this concept be first evaluated in the murine model and if successful, in a randomized human clinical trial."

We'll report back after the conference.