Final answer:
Cori Disease (Glycogen Storage Disease type III) involves a deficiency in debranching enzyme affecting liver and muscles, whereas Von Gierke Disease (Glycogen Storage Disease type I) is characterized by a lack of glucose-6-phosphatase, leading to liver and kidney issues and more severe symptoms like hyperuricemia.
Step-by-step explanation:
Differences between Cori Disease and Von Gierke Disease
Cori Disease and Von Gierke Disease are both glycogen storage diseases, but they differ in the specific enzyme deficiencies and bodily tissues affected. Cori Disease, also known as Glycogen Storage Disease type III, is characterized by a deficiency of the debranching enzyme amylo-1,6-glucosidase. This enzyme is crucial for the proper degradation of glycogen, and its deficiency leads to the accumulation of abnormal glycogen molecules, primarily affecting the liver and muscles.
On the other hand, Von Gierke Disease, or Glycogen Storage Disease type I, is caused by a deficiency in the enzyme glucose-6-phosphatase. This enzyme is essential for glucose production during gluconeogenesis and glycogenolysis. Its absence leads to the accumulation of glycogen and fat in the liver and kidneys, impairing the ability of these organs to release glucose into the bloodstream, leading to severe hypoglycemia.
While both Cori Disease and Von Gierke Disease can present with hepatomegaly (enlarged liver) and hypoglycemia, Von Gierke typically presents with more severe symptoms and complications, such as lactic acidosis, hyperuricemia, and hyperlipidemia, due to the liver and kidney involvement. Cori Disease sufferers often exhibit muscle weakness and cardiomyopathy due to muscle involvement.