Final answer:
Glycogen storage diseases lead to excess glycogen storage, particularly affecting heart, skeletal muscles, liver, and nervous system, creating symptoms like progressive muscle weakness. Increased glucose due to defective glycogen mobilization may also lead to fatty acid deposition in adipose tissues, while hyperinsulinism can result in hypoglycemic symptoms.
Step-by-step explanation:
Symptoms of glycogen storage diseases (GSDs) often extend beyond excess glycogen storage in the body. In Glycogen storage disease type-II, also known as Pompe disease, patients typically experience progressive muscle weakness, a condition referred to as myopathy. This affects various tissues including the heart, skeletal muscles, liver, and nervous system. In the context of metabolism, when the body has adequate ATP and glucose, it synthesizes glycogen for future energy needs.
However, in GSDs, there's a problematic build-up due to defective glycogen mobilization. Additional glucose not stored as glycogen can lead to increased fatty acid synthesis, which is deposited in adipose tissue. In the scenario of hyperinsulinism, excess insulin leads to hypoglycemia, showing symptoms like sweating, tremors, and fainting, which can be alleviated by glucose administration.