The addition of UDP-glucose to glycogen is catalyzed by an enzyme called glycoge

Question

The addition of UDP-glucose to glycogen is catalyzed by an enzyme called glycogen synthase. Glycogen breakdown is complex, but one enzyme called glycogen phosphorylase can remove glucose from the ends of glycogen molecules. Note that a single inorganic phosphate group is added to the molecule, breaking it into two pieces - a glycogen chain that is one glucose molecule shorter, and glucose 1-phosphate.

The reaction catalyzed by glycogen phosphorylase is shown below, and has a very large negative ?G. Note that the R shown on the glycogen molecules refers, in this case, to the rest of the long glycogen polymer. R is commonly used in representations of molecules to represent any molecule attached to the molecule of interest.

Glycogen synthesis and breakdown are most common in the liver and muscle. A very rare human disease is caused by a genetic deficiency of the liver glycogen phosphorylase enzyme. What molecule in the glycogen synthesis pathway is likely to accumulate the most in the liver and muscle cells of people with this disease?

- glucose 6-phosphate

- glycogen

- glucose

?? HO HO HO HPO.2 4 OH OH OH ?? OR ?? HO OPO 2 H? 3 OH ?? ?? ?? Glycogenn) Glucose 1-phosphate Glycogen(n-1) HMX Copyright The President and Fellows of Harvard College

Explanation / Answer

Glycogen

The gylycogen will not be broken down due to deficiency of glycogen phosphorylase. This disease is called as Her's disease. This leads to accumulation of glycogen in muscles and liver.

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