Glycogen Metabolism Pathways

Carbohydrate Metabolism

• The major pathways of carbohydrate metabolism start or end with glucose, involving utilization of glucose as energy, gluconeogenesis, glycogen storage, glycogen release, and pentose phosphate pathway.

Glycogen Overview

• Glycogen is the main storage carbohydrate in animals, similar to starch in plants.
• It is a branched polymer of α-D-glucose.
• Glycogen is primarily found in the liver and muscles, with small amounts in the brain.
• Glycogen synthesis and degradation are essential pathways of energy metabolism.

Glycogenolysis and Glycogenesis

• Glycogenolysis is the release of glucose from glycogen.
• Glycogenesis is the storage of glucose as glycogen.

Glycogen Storage Diseases (GSDs)

• GSDs are genetic diseases caused by defects in enzymes required for glycogen synthesis or degradation.
• Defects can result in abnormal glycogen structure or excessive accumulation of normal glycogen in specific tissues.
• Enzyme defects can affect single tissues (e.g., liver or muscle) or be more generalized, affecting multiple tissues (e.g., liver, muscle, kidney, intestine, and myocardium).
• The severity of GSDs ranges from fatal in infancy to mild, non-life-threatening disorders.

Carbohydrate Metabolism

• The major pathways of carbohydrate metabolism start or end with glucose, involving utilization of glucose as energy, gluconeogenesis, glycogen storage, glycogen release, and pentose phosphate pathway.

Glycogen Overview

• Glycogen is the main storage carbohydrate in animals, similar to starch in plants.
• It is a branched polymer of α-D-glucose.
• Glycogen is primarily found in the liver and muscles, with small amounts in the brain.
• Glycogen synthesis and degradation are essential pathways of energy metabolism.

Glycogenolysis and Glycogenesis

• Glycogenolysis is the release of glucose from glycogen.
• Glycogenesis is the storage of glucose as glycogen.

Glycogen Storage Diseases (GSDs)

• GSDs are genetic diseases caused by defects in enzymes required for glycogen synthesis or degradation.
• Defects can result in abnormal glycogen structure or excessive accumulation of normal glycogen in specific tissues.
• Enzyme defects can affect single tissues (e.g., liver or muscle) or be more generalized, affecting multiple tissues (e.g., liver, muscle, kidney, intestine, and myocardium).
• The severity of GSDs ranges from fatal in infancy to mild, non-life-threatening disorders.