The content of this website is intended for UK patients. Information concerning liver Glycogen Storage Disease management is based upon UK practice. As dietary management may vary in different countries, please speak to your metabolic team at the hospital for individualised advice for your GSD.
You may never have come across this term before, so here are some of the commonly asked questions about this molecule.
It is one of the main energy stores in your body.
Energy from carbohydrates (such as glucose) that is not for immediate use, gets stored as glycogen. When the body needs more energy, glucose can be released from the body’s stores.
It is stored in multiple locations, most notably in the liver and the muscles.
The liver’s glycogen stores are used to control the levels of glucose in the blood and this influences energy levels throughout the body. A liver GSD indicates an issue with this process.
Muscle glycogen stores are used to provide energy for the muscles.
Muscle glycogen storage disorders require very different management to liver GSDs. They are not discussed further on this website.
When you eat a meal that is rich in carbohydrates, the level of glucose in the blood starts to rise. A hormone called insulin will then be released.
Insulin removes excess glucose from the blood. This excess glucose is mainly placed in the liver glycogen stores.
When blood glucose levels start to drop, the body releases another hormone called glucagon. Glucagon causes the release of glucose from the glycogen stores.
This process does not work in GSD.