Exam 25: Glycogen Synthesis

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At the center of a glycogen molecule is:

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B

Hydrolysis of _______________ drives the formation of UDP-glucose.

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J

_______________ is the storage disease in which light exercise results in cramps.

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L

Glycogen synthase catalyzes the transfer of a glucose residue from UDP-glucose to one of the __________________ ends of glycogen.

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Explain why patients with McArdle disease do not accumulate lactate during light exercise.

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Glycogenin:

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Glucosyl units are added to the _______________ end, or terminal, of glycogen.

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_______________ is the activated form of glucose that is required for glycogen synthesis.

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Type I diabetes leads to ______________ in the liver.

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Synthesis of glycogen starts with the phosphate group transfer from UTP to _______________.

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The protein _______________ serves as the primer used by glycogen synthase.

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______________ results from a loss or inactivation of insulin receptors.

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There is rare disease in which a critical serine in muscle glycogenin is mutated to alanine, which results in what biochemical outcome?

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Explain the conversion between both a and b, and R and T forms of phosphorylase.

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_______________ dissociates from phosphorylase a in the T state.

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The glycogen branching enzyme moves a block of __________________ (or so) glucose residues to from a branch point at least four residues from a preexisting branch.

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Glycogen _______________ is inhibited by phosphorylase kinase.

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Which enzyme(s) is/are required to synthesize ɑ-1,4-glycosidic bonds in glycogen?

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Insulin binds to its receptor and causes what protein to be activated in the cytoplasm?

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What did Carl and Gerti Cori discover about the cause of von Gierke disease? Briefly explain how this information helps to explain the symptoms of the disease.

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