Glucose-6-phosphatase deficiency (G6PD), also known as von Gierke disease, is glycogen storage disease. It is an autosomal recessive disease occurring most frequently in males of African or Middle Eastern descent. G6PD is involved in many reactions, but we will focus on the following simplified pathway: The conversion of glycogen to glucose is called:
Glucose-6-phosphatase deficiency (G6PD), also known as von Gierke disease, is glycogen storage disease. It is an autosomal recessive disease occurring most frequently in males of African or Middle Eastern descent. G6PD is involved in many reactions, but we will focus on the following simplified pathway:
The conversion of glycogen to glucose is called:
Introduction
Carbohydrates, proteins, and fats are the three macronutrients that are present in our food. Carbohydrates serve as the major source of energy for the body. Carbohydrates are made up of sugar molecules called glucose which is used for producing energy in the form of ATP through a process called cellular respiration that takes place in the mitochondria of the cells. Two major hormones are involved in maintaining blood glucose homeostasis and they are insulin which is produced by the beta cells of the islets of Langerhans present in the pancreas and glucagon which is produced by the alpha cells of the islets of Langerhans present in the pancreas. Insulin helps the cells of the body to take up glucose and utilize it for energy. If a person is deficient in this hormone he develops a condition called diabetes. It is released when the blood glucose levels are high or the body is in a hyperglycemic state. Glucagon is the hormone that increases the blood levels of glucose when the body is in a glucose-deficient state or when the body is facing hypoglycemia.
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