A child has a history of hepatomegaly, hypoglycemia, seizures, especially on an empty stomach and in stressful situations. The child is diagnosed with Gierke disease. This disease is caused by the genetic defect of the following enzyme:

Answer options

1. Glucokinase
2. Glycogen phosphorylase
3. Amyloid-1,6-glycosidase
4. Glucose-6-phosphatase
5. Phosphoglucomutase

Explanation

Gierke’s disease is the most common of the glycogen storage diseases. This genetic disease results from a deficiency of the enzyme glucose-6-phosphatase, it impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis.