Glycogen storage disease type IX Glycogen storage disease type IX Glycogen storage disease type IX is a hereditary deficiency of glycogen phosphorylase kinase B that affects the liver and skeletal muscle tissue. It is inherited in an X-linked or autosomal recessive manner. The signs and symptoms in glycogen storage disease type IX include: Most of these signs and symptoms diminish as adulthood sets in. Glycogen storage disease type IX can be inherited via: The diagnosis of glycogen storage disease IX consists of the following: There are two types of this inherited condition, "glycogen storage disease IXa1" and "glycogen storage disease IXa2" that affect the liver
Glycogen-branching enzyme deficiency known as glycogen synthase. This bond may be broken by amylase when the body wishes to break down glycogen into glucose for energy. Glycogen branching enzyme is responsible for the required α-1,6-glycosidic bonds needed to start a branch off of these linear chains. These branches are important, as they provide additional "free ends" for linear chains of α-1,4-glycosidic bonds, which can then be broken down by amylase. This allows for glucose to be removed at a faster rate than if all glucose molecules were in a single chain with only two free ends on which amylase could attach. GBED is