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Glucose Storage Disease

Glucose storage disease, otherwise known as glycogen storage disease, falls under the category of a metabolic disorder according to the Association for Glycogen Storage Disease. Therefore, glucose storage disease makes it difficult for the body to break down food and convert it to energy. When glucose is stored, it's converted to glycogen in the body. Glycogen storage diseases specifically make it difficult for the body to store and use glycogen. There are many different types of glycogen storage disease.

  1. Type I Glyocgen Storage Disease

    • Twenty-five percent of glycogen storage disease cases in Europe and the United States are Type I Glycogen Storage Disease. For every 100,000 births, approximately one child will be diagnosed with Type I Glycogen Storage Disease. Symptoms of this disease include low blood sugar, or hypoglycemia, and an enlarged liver. With Type I Glycogen Storage Disease, the enzyme that's needed to break down glucose in the liver is actually missing. People with this condition have a difficult time keeping their blood sugar levels high according to the Association for Glycogen Storage Disease.

    Type II Glycogen Storage Disease

    • Type II Glycogen Storage Disease, also known as Acid Maltase Deficiency or Pompe Disease, is an autosomal recessive genetic disease that results from the maltase enzyme not functioning properly. Lysosomes are organelles in cells that help break down glucose with the help of maltase. The problem with Type II GSD is that lysosomes cannot break glycogen down into glucose because of the lack of maltase in the cell. Type II GSD can be treated with enzyme replacement therapy which helps replace maltase in the body. One of the first symptoms associated with this disease is lack of muscle strength according to the Association for Glycogen Storage Disease.

    Type III Glycogen Storage Disease

    • Type III Glycogen Storage Disease results from a deficiency of the activity of the glycogen debrancher enzyme (GDE) according to the Association for Glycogen Storage Disease. Glycogen debrancher enzymes help break glycogen down into glucose. Absence of this enzyme results in hypoglycemia or low blood sugar levels. With this disease, glycogen is only partially broken down which results in glycogen being stored in the organs of the body instead of in the liver.

    Type IV Glycogen Storage Disease

    • Type IV Glycogen Storage Disease is one of the most serious forms of GSD. Type IV GSD results in glycogen being formed that has extremely long outer branches because of the absence of a branching enzyme. As a result, glycogen is not stored in the organs of the body as much. However, glycogen that is stored is actually attacked by the body's immune system along with the tissues that the glycogen is stored in. This results in cirrhosis or scaring of the liver along with other organs and muscles, according to the Association for Glycogen Storage Disease. Type IV Glycogen Storage Disease can sometimes be treated with a liver transplant.

    Type V Glycogen Storage Disease

    • According to the Association for Glycogen Storage Disease, Type V Glycogen Storage Disease occurs when the phosphorylase enzyme is not present. Without this enzyme, glycogen stored in skeletal muscles cannot be released in the form of glucose to provide energy. People with Type V Glycogen Storage Disease have an extremely difficult time participating in physical activities especially anaerobic activities.

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