Glycogen Metabolism Flashcards

1
Q

Which parts of the body have the most blood glucose demand?

A

Brain and RBC. They both consume 80% of the 200g of glucose consumed by body per day.

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2
Q

Why is it necessary for our body to keep replenishing blood glucose levels?

A
  1. Our blood plasma only has 10g of glucose at once (around 5% of what our body needs)
  2. Glucose is absorbed from our intestines only 2-3 hours after a carbohydrate meal.
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3
Q

What are the symptoms of hypoglycemia?

A
  1. Compromised brain function (confusion and disorientation)
  2. Coma

Hypoglycemia: 2.5 mmol/dL (45 mg/dL)

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4
Q

How is glycogen stored in the cytosol?

A

As granules

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5
Q

Why is glycogen preferred over fat for energy breakdown?

A
  1. Muscles cannot mobilise fat as effectively as glycogen.
  2. Fatty acids cannot be metabolised under anaerobic conditions (G-6-P can enter glycolysis to give pyruvate, then lactose)
  3. Fatty acids cannot be converted back to glucose. (Pyruvate to Acetyl CoA is irreversible)
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6
Q

Which organ stores the mst glycogen per tissue mass?

A

Liver (10g/100g)

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7
Q

How much glycogen is stored in muscle and in liver?

A

Muscle: 300- 400g
Liver: 100g

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8
Q

When is muscle and liver glycogen depleted?

A

Muscle: Muscle contraction
Liver: After 12-18 hours of fasting

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9
Q

Where is the glucose added to the glycogen primer?

A

To the non reducing end by the alpha-1,4 glycosidic bond.

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10
Q

What is glycogenin?

A

Glycogenin is a glycoprotein with two polypepetide chains that acts as the glycogen primer.

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11
Q

What is the property of glycogen synthase?

A

It is a glycosyl transferase

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12
Q

What is the property of the branching enzyme?

A
  1. Transglycosylase (alpha 1,6 bond)
  2. Glucosidase (alpha 1,4 bond)
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13
Q

What are the properties of the debranching enzyme?

A

Glucosidase (alpha 1,6 bond)

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14
Q

Why can’t muscle glycogen regulate blood glucose level?

A

Blood lacks G-6-P phosphatase, hence it cannot dephosphorylate G-6-P to give free dlucose to release into the blood.

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15
Q

Which is the regulatory/ rate limiting step of glycogenesis?

A

Glycogen synthase

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16
Q

What is the regulatory/ rate limiting step for glycogenolysis?

A

Glycogen phosphorylase

17
Q

How does Glycogen phosphorylase work?

A

Breaks apha 1,4 bond and stops 3-4 units away from the branching point.

18
Q

What is a coenzyme for Glycogen phosphorylase?

A

PLP (Pyridoxine 5’ phosphate, derived from vitamin B6)

19
Q

Which are the primary and secondary messenger of glycogenesis and glycogenolysis regulation?

A
  1. Primary: Glucagon and Epinephrine
  2. Secondary: cAMP
20
Q

When do epinephrine and glucagon regulate glycogenesis and glycogenolysis?

A

In the state of starvation or hypoglycemia to sop glycogen synthesis and stimulate its breakdown.

21
Q

What are Glycogen Storage Diseases?

A

It is a term given to a group of inherited disorders with:
1. Deposition of abnormal type of glycogen
2. Deposition of abnormal quantity of glycogen
3. Failure to mobilise glycogen

22
Q

What is Glycogen Storage Disease Type 1 also called?

A

von Gierke’s Disease

23
Q

What is the cause of Glycogen storage disease Type 1?

A

Deficiency in Glucose 6 phosphatase

24
Q

What are the salient features of Glycogen Storage Disease Type 1?

A
  1. Hypoglycemia
  2. Hyperlipidemia
  3. Lactic Acidosis
  4. Ketosis
  5. Osteoporosis
  6. Hyperuricemia
  7. Increased infections and Mouth ulcers
  8. Liver enlargement
  9. Impaired growth and delayed puberty
25
Q

How is von Gierke’s disease diagnosed?

A

Liver biopsy

26
Q

What are suitable treatment options for von Gierke’s disease?

A
  1. Giving food at short intervals
  2. Nasogastric feeding at night