The following is a list of hereditary metabolic defects involving loss of single

ID: 31441 • Letter: T

Question

The following is a list of hereditary metabolic defects involving loss of single enzymes of catabolism, and a second list of possible consequences of such defects. Match each enzyme with its most likely consequence (only one).

lack of phosphoglucomutase

lack of UDP-glucose pyrophosphorylase

lack of triose phosphate isomerase

lack of phosphofructokinase

lack of glycogen phosphorylase kinase

F. lack of ?-1,6-glucosidase

1. impaired ability to obtain energy from carbohydrates.

2. lower than normal production of glucose 1-P from glycogenolysis.

3. inability to use either glycogen or galactose as an energy source

4. inability to use galactose as an energy source, with no effect on ability to use glycogen.

5. lethal; prevents use of carbohydrates for ATP production

6. inability to use glycogen as an energy source, with no effect on ability to use galactose.