Anna and Beth have decided to run the Great Ocean Road Marathon this year. Since they have never run a marathon before and don’t usually exercise much, they decide to start practicing early on in preparation.  

They go for a run one early morning and for the first 2-3 minutes everything goes well, but Beth suddenly feels intense cramping and pain in her muscles. They both stop and after resting for about 5 minutes Beth starts to feel better and starts running again.

The same thing happens each time they go for a run and Beth decides to visit a doctor. The doctor diagnoses her with McArdle’s disease and recommends she avoid strenuous exercise.

This disease is caused by a deficiency in the glycogen phosphorylase enzyme which is involved in glycogen breakdown.

Which glycogen phosphorylase is affected?

True or False?

Which of the following urea cycle product is a metabolic intermediate of the TCA cycle?

Amino acids that are converted to acetyl CoA and or acetoacetate are:

What molecule is added as a tag to mark the proteins for degradation?

A mouse born with a defect in the gene that produces trypsin inhibitor was seen to have pancreatitis.

Enter answer.

True or False?

Once the protein is tagged for degradation which protein complex is involved in the degradation of the marked protein?

True or False?

Describe how trypsinogen is activated and discuss the consequences of this activation. 

Enter answer.