Glycogen Metabolism Flashcards

1
Q

What is glycogen?

A

A storage polysaccharide made up of glucose units, characterized by extensive branching to store large amounts of glucose.

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2
Q

What enzyme adds glucose-1-phosphate molecules to glycogen?

A

Glycogen synthase.

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3
Q

What enzyme breaks down glycogen?

A

Glycogen phosphorylase.

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4
Q

What is glycogenolysis?

A

The breakdown of glycogen.

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5
Q

What is glycogenesis?

A

The synthesis of glycogen.

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6
Q

How is glycogenolysis regulated?

A

It is regulated by phosphorylation of glycogen phosphorylase.

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7
Q

What stimulates glycogen breakdown in the liver during fasting?

A

Glucagon stimulates the release of glycogen breakdown, increasing blood glucose levels.

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8
Q

What is the role of protein kinase A in glycogen metabolism?

A

It phosphorylates phosphorylase kinase, activating glycogen phosphorylase and promoting glycogen breakdown.

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9
Q

What happens when glycogen phosphorylase is phosphorylated?

A

It converts from its low activity form (phosphorylase b) to its high activity form (phosphorylase a).

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10
Q

What is the effect of high glucose concentration on liver glycogen phosphorylase?

A

Glucose acts as an allosteric inhibitor, preventing glycogen breakdown.

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11
Q

What activates skeletal glycogen phosphorylase?

A

Allosteric activation occurs in rapidly contracting muscles due to high AMP concentration.

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12
Q

How does calcium affect glycogen breakdown?

A

Calcium release stimulates muscle contraction and glycogen breakdown.

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13
Q

What is the reciprocal regulation of glycogen metabolism?

A

Protein kinase A phosphorylates glycogen synthase, converting it to its inactive form, thus inhibiting glycogen synthesis while promoting breakdown.

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14
Q

How is protein phosphatase 1 (PP1) regulated in the fasted state?

A

PP1 can be downregulated by PKA-dependent phosphorylation mechanisms.

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15
Q

What activates glycogen synthase in the fed state?

A

Increased blood glucose and insulin release activate glycogen synthase via protein kinase B.

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16
Q

What is the role of insulin in glycogen metabolism?

A

Insulin activates protein phosphatase 1, promoting glycogen synthesis and inhibiting glycogen breakdown.

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17
Q

Why does a deficiency in muscle glycogen phosphorylase cause mild symptoms?

A

Muscle glycogen is used for contraction, and deficiency leads to cramps without glucose release into the blood.

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18
Q

What is the consequence of liver glycogen phosphorylase deficiency?

A

It can lead to severe hypoglycemia and potential coma due to inadequate glucose release.

19
Q

What are lipids?

A

More reduced than carbohydrates, generally insoluble in water, and release more energy when oxidized.

20
Q

What are ketone bodies?

A

Water-soluble fuel molecules, such as acetoacetate and hydroxybutyrate, derived from acetyl-CoA.

21
Q

Where do lipids in our body come from?

A

From diet, storage, utilization as energy, or synthesis in the body.

22
Q

What is the function of lipoproteins?

A

They transport lipids, consisting of a neutral core of TAGs and cholesterol esters, surrounded by phospholipids and apolipoproteins.

23
Q

What is ApoB-100?

A

A protein part of a lipoprotein involved in cholesterol uptake by receptor-mediated endocytosis.

24
Q

What are chylomicrons?

A

A type of lipoprotein mainly consisting of triacylglycerols and proteins like ApoB-48, ApoC-II, and ApoE.

25
What is the role of ApoC-II?
It activates lipoprotein lipase (LPL), which hydrolyzes TAGs in chylomicrons into free fatty acids and glycerol.
26
What is the process of dietary lipid processing?
Involves solubilization, breakdown, packaging, transport, and release/uptake of cargo at target tissues.
27
How are lipids solubilized?
Using bile salts in the gut, which make fats more accessible to enzymes like lipases.
28
Where does the formation of chylomicrons begin?
In the endoplasmic reticulum of intestinal epithelial cells.
29
What happens to nascent chylomicrons as they enter the blood?
They mature by acquiring ApoE and ApoC-II, enabling them to deliver lipid cargo to tissues.
30
How do tissues uptake lipids from TAG-rich lipoproteins?
LPL hydrolyzes TAGs, releasing free fatty acids for energy or storage and glycerol for gluconeogenesis.
31
Where does fatty acid biosynthesis occur?
In the cytoplasm, mainly in the liver and adipose tissue.
32
What is the first step in fatty acid biosynthesis?
Carboxylation of acetyl CoA to form malonyl CoA, catalyzed by acetyl CoA carboxylase.
33
What catalyzes the second step of fatty acid synthesis?
A multi-enzyme complex called fatty acid synthase (FAS).
34
What is the regulation of acetyl CoA carboxylase via hormones?
Glucagon inactivates it via phosphorylation, while insulin activates it through protein phosphatase 2A.
35
How is acetyl CoA carboxylase regulated by dimer/polymer transition?
The high activity form is a polymer, while the low activity form is a dimer, influenced by allosteric factors.
36
Where does NADPH for fatty acid synthesis come from?
It is produced in the pentose-phosphate pathway in the liver and adipose tissue.
37
Where does the acetyl CoA for fatty acid synthesis come from?
Partly from diet and from oxidizing excess carbohydrates.
38
How are fats transported and stored in response to feeding?
Acetyl CoA is packaged into triacylglycerols, which are then assembled into VLDL for release into the blood.
39
How are fatty acids mobilized from adipose tissue?
Catecholamines activate PKA, which activates perilipin and lipases to break down TAGs into free fatty acids.
40
What is B-oxidation of fatty acids?
A process occurring in the mitochondria that removes 2 carbons per cycle, producing NADH and FADH2.
41
What is the first step in B-oxidation of fatty acids?
Fatty acids are linked to coenzyme A in the cytoplasm via acyl CoA synthase.
42
What is the regulatory step in transporting fatty acyl CoA into the mitochondria?
Carnitine acyltransferase 1 is inhibited by malonyl-CoA, preventing fatty acid oxidation when synthesis is active.
43
What occurs in each round of B-oxidation?
A series of 4 reactions generates 1 NADH, 1 FADH2, and 1 acetyl CoA, shortening the fatty acid chain by 2 carbon atoms.
44
How else is B-oxidation of fatty acids regulated?
By inhibiting enzyme activity with high energy signals, controlling FA availability, and regulating entry into mitochondria.