What are the types of glycogen storage diseases?
What are the types of glycogen storage diseases?
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)
What are the symptoms of glycogen storage disease?
General symptoms of GSD may include:
- Not growing fast enough.
- Not feeling comfortable in hot weather (heat intolerance)
- Bruising too easily.
- Low blood sugar (hypoglycemia)
- An enlarged liver.
- A swollen belly.
- Weak muscles (low muscle tone)
- Muscle pain and cramping during exercise.
What happens when there is too much glycogen in the body?
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.
How can I reduce my liver glycogen?
refer to a number of studies that suggest that ingestion of carbohydrates, particularly glucose or sucrose (glucose-fructose) during exercise can attenuate liver glycogen depletion. It is believed that consuming 1.2g/kg of carbohydrate during recovery is ideal for rapid repletion.
What lysosomal storage diseases do you know?
Some of the most common lysosomal storage disorders include:
- Gaucher disease: Gaucher disease often causes spleen and liver enlargement, blood problems and bone issues.
- Fabry disease: This disorder often causes severe burning pains in hands and feet and, in some cases, a distinctive skin rash on the legs.
How is excess glucose stored in the liver?
Following consumption of food, excess glucose can be stored within the liver as glycogen. This is stimulated by insulin release. Around 100g of glycogen is stored in the liver (300g is stored in skeletal muscle). The synthesis of glycogen occurs in the following steps:
How does glycogen storage disease affect the body?
Glycogen storage disease (GSD) is a rare condition that changes the way the body uses and stores glycogen, a form of sugar or glucose. Glycogen is a main source of energy for the body.
What causes the accumulation of glycogen in the liver?
It is caused by deficient activity of the glycogen-branching enzyme (GBE), resulting in accumulation of abnormal glycogen in the liver, muscle, and/or other tissues. In most affected individuals, symptoms and findings become evident in the first few years of life.
What are the symptoms of lipid storage disease?
Symptoms may begin early in life or in adulthood and include enlarged liver and grossly enlarged spleen, which can rupture and cause additional complications. Skeletal weakness and bone disease may be extensive. People in this group usually bruise easily due to low blood platelet count. They may also experience fatigue due to anemia.