Glycogen Metabolism Pathways

Study Notes

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 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.

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.

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 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.

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.