Since GS, GYG, and GBE are the main enzymes that regulate glycogen synthesis, dysregulation of their enzymatic activities disrupts the homeostasis of glycogen metabolism, leading to glycogen storage diseases (GSDs) that are characterized by accumulations of excessive or abnormal glycogen, termed polyglucosan bodies (PB)15. This evidence concerns the gene GYG1 and Glycogen storage disease due to glycogenin deficiency.