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32 Cards in this Set
- Front
- Back
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What types of bonds are found in glycogen? |
1. a1-4 2. a1-6 |
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Where is glycogen found? |
1. Liver 2. Skeletal mucle 3. Heart |
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What is the first step of glycogenesis? |
1. Glucose must be added as activated UDP-glucose to the glycogen |
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What enzyme adds activated UPD-glucose to glycogen? What is the primer for this step? |
1. Glycogen synthase 2. Preexisting glycogen molecule |
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What converts G6P to G1P in glycogenesis? |
1. Phosphoglucomutase |
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What enzyme adds the branching a1-6 linkages in glycogen? |
1. amylo-1-4, 1-6-transglucosidase |
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What enzymes makes UDP-glucose from G1P and UTP? |
1. UDP-glucose pyrophosphorylase |
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What enzyme removes glucose units from the glycogen polymer in glycogenolysis? |
1. Glycogen phosphorylase |
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What is the role of debranching enzyme in glycogenolysis? What is the activity of this enzyme? |
1. Moves a trios from the branch point to another branch 2. a-1,6-glucosidase activity--- removes the branched glucoyl residue |
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How does phosphorylase participate in glycogenolysis? |
1. Removes glucose residues until there are only 4 glucose residues left after branch point |
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What is the effect of insulin on glycogenolysis and glycogenesis? |
1. Stimulates glycogenesis 2. Inhibits glycogenolysis 3. In liver and muscle |
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What is the effect of glucagon on glycogenolysis and glycogenesis? |
1. Inhibits glycogenesis 2. Stimulates glycogenolysis 3. In liver |
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What is the effect of epi on glycogenolysis and glycogenesis? |
1. Stimulates glycogenolysis in liver and muscle |
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What class of reactions is responsible for regulation of glycogenolysis and glycogenesis? |
1. Phosphorylation/dephosphorylation |
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What are the two forms of glycogen phosphorylase? What is the difference between the two? |
1. Active phosphorylase-a 2. Inactive phosphorylase-b 3. The enzyme is active when phosphorylated |
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What enzyme is responsible for activation/deactivation of glycogen phosphoylase? |
1. Phosphorylase kinase |
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What is in charge of phosphorylase kinase regulation? |
1. Phosphorylation |
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What enzyme deactivates glycogen phosphorylase? |
1. Phosphoprotein phosphatase |
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What are the two forms of glycogen synthase? |
1. Glycogen synthase-a-- active and dephosphorylated 2. Glycogen synthase-b--- inactive and phosphorylated |
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What is the MOA of glucagon in the liver? |
1. Increase cAMP |
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What is the MOA of epinephrine in the liver? |
1. Increase cAMP 2. Increased cAMP inhibits glycolysis and stimulates gluconeogensis 3. Also increases Ca |
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What is the MOA of epinephrine in muscle? |
1. Increase cAMP 2. Increased cAMP stimulates glycolysis |
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What enzyme is deficient in GSD0a? |
1. Glycogen synthase |
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What are the manifestations of GSD0a? |
1. Hypoglycemia 2. Early death 3. Hyperketonia 4. Low blood lactate and alanine |
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What enzyme is deficient in Cori disease? |
1. Liver and muscle debranching enzyme |
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What are the manifestations of Cori disease? |
1. Infant hepatomegaly and myopathy |
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What enzyme is deficient in Andersen disease? |
1. Branching enzyme GSD4 |
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What are the manifestations of Andersen disease? |
1. Hepatosplenomegaly 2. Cirrhosis |
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What enzyme is deficient in McArdle disease? |
1. Phosphorylase GSD5 |
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What are the manifestations of McArdle disease? |
1. Exercise-induced cramps and pain 2. Myoglobinuria |
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What enzyme is deficient in Hers disease? |
1. Liver phosphorylase GSD6 |
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What are the manifestations of Hers disease? |
1. Hepatomegaly 2. Mild hypoglycemia 3. Hyperlipidemia and ketosis 4. Improvement with age |
