Glycogen storage disease type IX with enlarged livers. 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
Glycogen many respects serves as a countersignal to insulin. In response to insulin levels being below normal (when blood levels of glucose begin to fall below the normal range), glucagon is secreted in increasing amounts and stimulates both glycogenolysis (the breakdown of glycogen) and gluconeogenesis (the production of glucose from other sources). Muscle cell glycogen appears to function as an immediate reserve source of available glucose for muscle cells. Other cells that contain small amounts use it locally, as well. As muscle cells lack glucose-6-phosphatase, which is required to pass glucose into the blood, the glycogen they store is available solely