Metabolic Myopathies

Paper Summary:

Carbohydrates and fatty acids are major energy sources for muscle activity. Glycogen is stored in muscle tissue to allow quick conversion into glucose or pyruvate for use during times of exertion. However, these storage systems do not always function the way they should. Muscle glycogenoses is a group of glycogen storage diseases affecting muscle tissue. This paper details the various types of muscle glycogenoses, their respective pathways, and the physiological effects.

From the Paper:

"The blockage prevents patients from being able to adequately store properly packaged glycogen. The glycogen produced in the pathway lack adequate branching and results in liver and spleen symptoms. Further down the glycogenesis pathway, normally the resulting glycogen can be directly reprocessed into glucose through Acid Maltase. Glycogenosis Type II affects this Acid Maltase enzyme. Glycogen which enters the lysosome to be broken down to glucose never leaves the cell. Instead this metabolic error accumulates glycogen in the lysosomes critically affecting cellular functions."

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