Glycogen Storage Disorders
What is Glycogen Storage disease?
Glycogen storage diseases (GSDs) are a group of inherited
genetic disorders that cause glycogen to be improperly formed or
released in the body. They are characterized by the accumulation of
abnormal amounts or types of glycogen in tissues.
Glucose is a simple sugar, which is a form of carbohydrate. It
is found in many foods and is the main source of energy for our
bodies. Glycogen is the storage form of glucose in our bodies.
Glycogen storage diseases are due to either a deficiency or
blockage of an enzyme that is important in converting glucose to
glycogen so it can be stored in the body for later use. The main
places glycogen is stored in the body includes the liver and muscle
The main types of GSDs are categorized by number and name. They
Type I - Von Gierke disease, defect in glucose-6-phosphatase,
this is the most common type of GSD, and accounts for 90% of all
from: http://www.mda.org.au/Disorders/Metabolic/GSDIII.asp on
This page was reviewed 30 Mar 2016