What causes glycogen accumulation?
When the body needs more energy, certain proteins called enzymes break down glycogen into glucose. They send the glucose out into the body. When someone has GSD, they are missing one of the enzymes that breaks down glycogen. When an enzyme is missing, glycogen can build up in the liver.
Why is glycogen accumulation bad?
The inability to properly release glucose from glycogen can result in the abnormal accumulation of glycogen. In the liver (e.g., GSD type 3b and 3d) this can result in hepatomegaly with the potential for cirrhosis.
What are the two types of glycogen?
Types of Glycogen Storage Disease
- Type I (Von Gierke disease) – this is the most common type of glycogen storage disease, and accounts for 90% of all glycogen storage disease cases.
- Type II (Pompe’s disease, acid maltase deficiency)
- Type III (Cori’s disease)
- Type IV (Andersen’s disease)
- Type V (McArdle’s disease)
Is glycogen toxic?
Too much glycogen and fat stored within a cell can be toxic. This buildup damages organs and tissues throughout the body, particularly the liver and kidneys, leading to the signs and symptoms of GSDI.
What happens excess glycogen?
When glucose is in excess, the body stores it away in the form of glycogen in a process stimulated by insulin. Glycogen is a large highly branched structure, made from lots of glucose molecules linked together. When required, glycogen can be easily and rapidly broken down again to form glucose.
Is glycogen storage disease life threatening?
Because they affect so many organ systems, GSD Type II (Pompe’s disease) and GSD Type IV (Andersen’s disease) are very hard to treat and can be fatal. Research into enzyme replacement therapy and gene therapy is promising, which may improve the outlook for the future.
What type of doctor treats glycogen storage disease?
After diagnosis, children with GSD are usually cared for by several specialists, including specialists in endocrinology and metabolism. Specific dietitians with expertise in this disease should be involved.
What’s glycogen used for?
This stored form of glucose is made up of many connected glucose molecules and is called glycogen. When the body needs a quick boost of energy or when the body isn’t getting glucose from food, glycogen is broken down to release glucose into the bloodstream to be used as fuel for the cells.
What is glycogen also called?
Similar to starch, the glycogen is a complex carbohydrate that stores excess glucose. It is sometimes referred to as “animal starch.” That is because the amylopectin constituent of plant starch is similar in composition and structure with the polysaccharide constituent of glycogen.
What does glycogen do in the body?
Does glycogen turn to fat?
If more glucose is consumed than can be stored as glycogen, it’s converted to fat for long-term storage of energy.