1) What is the normal structure of hepatic glycogen?

The liver is essential for monitoring and maintaining a relatively constant

level of glucose in the bloodstream. Conditions leading to glucose
concentrations being too high or too low are very detrimental. It was noted
that the liver is involved in gluconeogenesis in the Cori cycle and it should
come as no surprise that the liver is involved in glycogen breakdown and
synthesis because these pathways allow the liver to remove glucose from the
bloodstream for glycogen synthesis when blood glucose is high and to release
glucose into the bloodstream from glycogen breakdown when blood glucose
levels are too low. The enzyme glucose-6-phosphatase (G6Pase) catalyzes the
last step of gluconeogenesis - conversion of G6P to glucose + phosphate.
This enzyme is necessary also for release of glucose into the bloodstream
from glycogen metabolism (glycogen -> G1P -> G6P -> Glucose). It is
interesting to note that G6Pase is ABSENT FROM MUSCLE. This is because
muscle does NOT export glucose. the liver, on the other hand, DOES export
glucose and thus has abundant supplies of the enzyme.
As a meal containing carbohydrates or protein is eaten and digested, blood
glucose levels rise, and the pancreas secretes insulin. Blood glucose from
the portal vein enters liver cells (hepatocytes). Insulin acts on the
hepatocytes to stimulate the action of several enzymes, including glycogen
synthase. Glucose molecules are added to the chains of glycogen as long as
both insulin and glucose remain plentiful. In this postprandial or "fed" state,
the liver takes in more glucose from the blood than it releases.

2. How can this article help our patient in the case?

The article can help our patient determine the possible complications of the
mutation in the G6PC gene. It could be used as a precautionary measure and have
the patient regularly checked by specialists to prevent acquiring a life threatening
complications.