Glycogen-Rosenberg Flashcards

1
Q

Which organ is the main source of Glycogen during fasting?

A

The Liver

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2
Q

Regarding Glycogen, what is the fundamental difference between the liver and muscle?

A

The liver liberates glycogen to yield a higher concentration of blood glucose, while the muscle retains glycogen for muscle energy.

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3
Q

What are the two types of tissue that are insulin-sensitive “insulin dependent”?

A

Adipose and muscle tissue. “most abundant tissues in the body”.

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4
Q

How do muscle and adipose tissue bring glucose into the cell after a meal?

A

Insulin binds a receptor tyrosine kinase –> Protein kinase b activated –> endosomes which contain glut-4 fuses with cell membrane –> glucose transport into cell.

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5
Q

How do muscle and adipose tissue bring glucose into the cell during fasting conditions?

A

intracellular AMP concentration increases –> AMPK activation –> GLUT-4 membrane recruitment –> glucose transport into cell.

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6
Q

Which intracellular hepatic enzyme phosphorylates glucose?

A

Glucokinase

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7
Q

Which intracellular enzyme phosphorylates glucose?

A

Hexokinase

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8
Q

What would the activity be “high or low” of glucokinase in starving conditions?

A

Low

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9
Q

What would the activity be “high or low” of glucokinase in a diabetic patient?

A

Low

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10
Q

What would the activity be “high or low” of glucokinase after a meal?

A

High

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11
Q

What would the activity be “high or low” of glucokinase in a patient on a low carbohydrate diet?

A

Low

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12
Q

Which enzyme would work better for the brain: Hexokinase or Glucokinase and why?

A

Hexokinase because it has a higher affinity for glucose.

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13
Q

Under fasting conditions would glucose be phosphorylated or dephosphorlyated in the liver?

A

Dephosphorylated to liberate glucose into the blood

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14
Q

Most cells utilize GLUT-2 only to internalize glucose. Which cells also utilize GLUT-2 to transport glucose out of the cell?

A

Hepatocytes

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15
Q

Glycogen synthase

A

transfers glucose to glycogen primer that has “glycogenin”.

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16
Q

Does it require energy to make glycogen?

A

Yes, 2 ATP. One to phosphorylate glucose and one to make UTP from UDP.

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17
Q

Glycogen + Glycogen phosphorylase =

A

G-1-P via inorganic phosphorolysis @ non-reducing end of glycogen molecule.

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18
Q

Which enzyme is unique to the liver that takes G-6-P to Glucose?

A

Glucose-6- Phosphotase

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19
Q

Two types of Regulation:

A

Allosteric and Hormonal

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20
Q

Allosteric

A

Enzyme has a site which changes the catalytic activity. Concentration levels are the key.

21
Q

When is Glycogen phosphorylase structally active?

A

When it is phosphorylated.

22
Q

When are Glycogen phosphorylase and Glycogen synthase phosphorylated? During fasting or after a meal?

A

Fasting.

23
Q

When is Glycogen synthase structally active?

A

When it is dephosphorylated.

24
Q

Remember: When we fast we…….

A

Phosphorylate!!!!!

25
Q

Glucagon is secreted when blood glucose increases or decreases?

A

Decreases

26
Q

T/F Glucagon works only on the liver.

A

True. Makes liver break down glycogen to release glucose into the blood.

27
Q

T/F Epinephrine targets the liver and muscle.

A

True.

28
Q

Which 3 enzymes Deactivate Glycogen synthase-A?

A

PKA, GSK-3, Phosphorylase Kinase

29
Q

What would be favored in the liver by increased intracellular levels of epinephrine? glycogen breakdown or synthesis?

A

Breakdown.

30
Q

What would be favored in the liver by increased intracellular levels of glucagon? glycogen breakdown or synthesis?

A

Breakdown.

31
Q

4 functions of insulin:

A

limit the production of cAMP

activate hepatic protein phosphatases

activate the phosphodiesterase that converts cAMP to AMP.

Inactivates glycogen synthase kinase-3.

32
Q

When Insulin:Glucagon ratio is high what would be permitted?

A

Glycogen synthesis.

33
Q

How does glycogen inhibit its own formation?

A

by inhibiting dephosphorylation of glycogen synthase by phosphoprotein phosphatase or by making the active form of the synthase a better substrate for protein kinase A.

34
Q

Glycogen Storage Von Gierke’s disease is characterized by a defect in which enzyme?

A

Glucose-6-phosphotase

35
Q

Glycogen Storage Cori’s Disease is characterized by a defect in which enzyme?

A

Amylo-1,6-glucosidase (Debranching Enzyme)

36
Q

Glycogen Storage Anderson’s disease is characterized by a defect in which enzyme?

A

Branching Enzyme “Alpha-1,4 –> Alpha-1,6”

37
Q

Glycogen Storage Mc Ardle’s disease is characterized by a defect in which enzyme?

A

Glycogen phosphorylase

38
Q

Glycogen Storage Hers’s disease is characterized by a defect in which enzyme?

A

Glycogen phosphorylase

39
Q

What are Clinical features of Von Gierke’s disease?

A

Massive enlargement of the liver, Failure to thrive, Severe hypoglycemia, ketosis, hyperuricemia, hyperlipemea?

40
Q

What are clinical features of Cori’s disease?

A

Like Von Gierkes but milder.

41
Q

What are clinical features of Anderson’s Disease?

A

Progressive cirrhosis of the liver. Liver failure causes death usually before age two

42
Q

What are clinical features Mc Ardle’s disease?

A

Limited ability to perform strenuous exercise due to muscle cramps. Otherwise patient is normal and well developed.

43
Q

What are clinical features of Her’s Disease?

A

Like type 1 but milder.

44
Q

What is glycogen’s structure and concentration like in Von gierkes disease?

A

Increased amount and Normal structure

45
Q

What is glycogen’s structure and concentration like in Cori’s disease?

A

Increased amount and short outer branches

46
Q

What is glycogen’s structure and concentration like in Anderson’s disease?

A

Normal amount with very long outer branches

47
Q

What is glycogen’s structure and concentration like in Mc Ardles disease?

A

Moderately increased amount with normal structure

48
Q

What is glycogen’s structure and concentration like in Her’s Disease?

A

Increased amount.