section 6 in class question Flashcards

1
Q

Which of the following leads to an increased rate of glycogen breakdown in skeletal muscle?

A. AMP
B. epinephrine
C. epinephrine and AMP
D. glucagon
E. insulin
A

c

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2
Q

What is the purpose for converting glucose 1-phosphate to UDP-glucose prior to glycogen synthesis?

A. It allows glycogen formation to be reversible.
B. It ensures that multiple glucose carbons don’t react with the glycogen molecule.
C. It increases the reactivity of the glucose molecule.
D. It makes glycogen formation irreversible.
E. It results from insulin stimulation of the glycogen synthase.

A

c

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3
Q

the parents of a 3 month y/o boy bring him to the doctor for lethargy, swollen abdomen. Boy has massive hepatomegaly and enlarged kidneys with a normal spleen and heart. Lab studies are notable for hypoglycemia. Biopsy of muscle and liver show excess glycogen in the liver tissue but not in the muscle. What is happening?

A. glucokinase absent in liver tissue
B. Glucose 6 phosphatase absent in the liver tissue
C. Lack of glycogen synthase in muscle tissue
D. lack of phosphorylase in liver tissue

A

b Glucose 6 phosphatase absent in the liver tissue

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4
Q

Which enzyme commits glucose to the pentose phosphate pathway?

A. phosphofructose kinase-1
B. hexokinase or glucokinase (depending on the tissue)
C. glucose 6-phosphate dehydrogenase
D. glucose 6-phosphatase

A

c

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5
Q

Which of the following pathways will be adversely affected by a glucose-6-phosphate dehydrogenase deficiency?

Fatty acid synthesis
Cholesterol synthesis
Glutathione reduction
All the listed pathways would be affected.

A

d

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6
Q

A male patient who is fructose intolerant due to an Aldolase B deficiency has asked you whether his sperm cells will be able to obtain the necessary fructose to support motility. What will you tell him?

A. no - because sperm cells are not exposed to dietary fructose.
B. no – because dietary fructose must be processed before delivery to sperm cells.
C. you don’t know offhand, you will have to measure this.
D. yes – because sperm cells can make fructose from glucose as needed.
E. yes – because sperm depend on glucose as the sole energy source.

A

d

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7
Q

A long distance runner who is planning to run a marathon decides to add fructose to the replacement fluid she will be using during the race. Which of the following statements regarding fructose best describes the fate of this sugar?

it enters the glycolytic pathway as fructose 6 phosphate in the liver

it is converted to uridine diphosphate (UDP)-fructose and then epimerized to UDP glucose

it is metabolized by a pathway other than the glycolytic pathway

it is metabolized in the liver by an aldolase that recognizes fructose 1-phospahte

it is phosphorylated by phosphofructokinase

A

d

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8
Q
If a person has a phosphoenolpyruvate carboxykinase (PEPCK) defect, which of the following cannot be used as starting material for gluconeogenesis?
A. alanine
B. glycerol
C. lactate
D. neither alanine nor lactate
E. none of the above
A

d

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9
Q

A 25-year old male is found in a semicomatose state and is taken to the emergency department by his friends. The friends report that the patient has not eaten any food for the past 5 days. Which of the following substances is the most likely source of glucose for the patient’s blood?

A. Pyruvate from glycolysis in the liver
B. Alanine from muscle tissue
C. Glycerol from adipose tissue
D. Fatty acids from adipose tissue
E. Lactate from skeletal muscle
A

c

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10
Q

Fatty acids are used more than glucose by tissues with high energy demands. Why is this?

a. Fatty acid oxidation can be used by more tissue types than glycolysis.
b. The compartmentalization of fatty acid oxidation in mitochondria is more efficient for the electron transport chain and ATP synthase.
c. A typical fatty acid produces about 50 ATP molecules, whereas a glucose molecule yields about 25 ATP molecules.
d. A typical fatty acid yields about100 ATP molecules whereas a glucose molecule yields only about 25 ATP molecule.

A

d

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11
Q

A patient has an enzyme imbalance, such that her cells have a chronically high level of malonylCoA. Which of the following would be a likely consequence of this imbalance?

a. Her acetylCoA levels would be vanishingly small.
b. Her cells will make excess ketone bodies
c. Fatty acid oxidation will be inhibited in her cells
d. Fatty acid oxidation will be increased in her cells

A

c

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12
Q

A patient has a defect in her carnitine-palmatoyl transferase I enzyme that makes this enzyme inactive. Which of the following would be a likely consequence of this defect?

His mitochondria will oxidize newly synthesized fatty acids
His fatty acid oxidation will take place in the cell cytoplasm
He will generate significantly less ketone bodies during fasting.
His fatty acid oxidation will be upregulated to compensate.

A

c

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13
Q

Which enzyme commits acetyl CoA to fatty acid synthesis?

a. Pyruvate dehydrogenase
b. Acetyl CoA carboxylase
c. Citrate lyase
d. Malic enzyme

A

b

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14
Q

Which enzyme if deficient in a patient would essentially prevent fatty acid synthesis?

a. Pyruvate dehydrogenase
b. Acetyl CoA carboxylase
c. Citrate lyase
d. Malic enzyme
e. phosphoenolypyruvate carboxy kinase

A

b

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15
Q

A patient has a disorder in his carnitine-palmitoyl tranferase I enzyme such that it does not recognize malonyl CoA. How will this defect affect fatty acid metabolism in his liver?

His cells will no longer oxidize fatty acids for energy.

His cells will likely oxidize newly synthesized fatty acids.

His liver will store excess fatty acids leading to fatty liver syndrome.

Fatty acyl CoA will not be converted to fatty acyl-carnitine by CPT-1

A

b

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16
Q

A young child presents with a metabolic disorder manifesting as an inability to synthesize fatty acids. Specific clinical tests indicate that the enzymes in the fatty acid synthesis pathway are all present and functional, and cytoplasmic acetyl CoA and malonyl CoA levels are normal. A defect in which enzyme could cause this problem?

a. Acetyl CoA carboxylase
b. Carnitine palmitoyl transferase I
c. Citrase lyase
d. Glucose 6-phosphate dehydrogenase

A

d, found in the PPP and responsible for NADPH production used to synthesize fatty acids

17
Q

A patient refuses to consume linoleic acid. How can this patient meet his body’s need for arachidonic acid?

a. His body will simply synthesize it de novo.
b. He can consume α-linolenic acid to synthesize linoleic acid.
c. He can consume DHA instead.
d. He can consume EPA instead.
e. He can consume arachidonic acid instead.

A

e

18
Q

Glycerol can serve as a starting point for triacylglycerol synthesis in liver but not in adipose tissue. What is the starting point for triacylglycerol synthesis in adipose tissue?

a. 1,2-diacylglycerol
b. phosphatidic acid
c. pyruvate
d. dihydroxyacetone phosphate

A

d

19
Q

An obese teenager presents with a metabolic disorder manifesting as an inability to liberate free fatty acids from triacylglycerol stores in adipose tissues. Clinical tests show that the patient is able to digest, absorb, utilize and store dietary triacylglcerols and fatty acids normally. However, following a fast and a glucagon challenge, free fatty acids do not appear in the blood stream. A defect in what enzyme could cause this problem?

a. Acetyl CoA carboxylase
b. Hormone sensitive lipase
c. Lipoprotein lipase
d. Lecithin-cholesterol acyltransferase

A

b

20
Q

A patient is very concerned that his adipose cells cannot import glycerol from the blood, since he knows glycerol is the backbone of triglycerides. What can you tell him that will allay his concerns?

a. His glycerol kinase will supply all the glycerol needed by adipose cells .
b. Adipose cells do not synthesize triglycerides: liver does this.
c. His adipose cells can use DHAP for TG synthesis instead of glycerol.
c. His adipose cells only use glycerol for gluconeogenesis.
d. His adipose cells will synthesize the glycerol needed for triglyceride formation.

A

c

21
Q

A patient has a PEPCK defect in his adipose cells. What will be the effect of this defect on adipose cell function?

a. Glycerol in his adipose cells will not get phosphorylated to support TG synthesis.
b. His adipose cells will not bind VLDL or chylomicrons properly.
c. His adipose cells will have a decreased ability to synthesize DHAP needed for TG formation.
c. His adipose cells will not be able to carry out gluconeogenesis.
d. His adipose not export free glycerol.

A

C

22
Q

A patient has a disorder in her adipose cell Lipoprotein Lipase (LPL) enzyme, where the enzyme does not recognize apoC-II on VLDL or chylomicron particles. Which of the following would be a likely consequence of this disorder?

a. Her adipose cells will be much smaller than normal.
b. Her blood lipoprotein level will be much higher than normal.
c. Her blood vessels will have increased level of fatty streaks.
d. Her liver may have more fatty deposits than normal.
e. All of the listed effects are likely consequences.

A

E

23
Q

Statin drugs are inhibitors of the enzyme HMG-CoA reductase and are often used for patients that have chronically high cholesterol levels in the blood. Which of the following would be an immediate change in cells for a patient on statin drugs?

The amount of mevalonate in the cell should decrease.

The amount of acetyl CoA in the cell will decrease (should increase)

More lanesterol will be converted to cholesterol to compensate.

The free isoprenes will be converted to triglyceride.

A

A

Not C, would be shut down
Not D, no oxygen so this would not happen

24
Q

A patient has an unusually low level of cholesterol in her blood (lipoproteins) and cells. Which of the following enzymes, if deficient, could account for this?

SREBP Cleavage Activating Protein (SCAP)

Acetyl CoA Carboxylase

Malic Enyme

LDL Receptor

Phosphoenolpyruvate carboxykinase

A

A

25
Q

Why does intracellular cholesterol regulate the number of LDL receptors on the cell surface?

a. To control the number of LDL particles that are synthesized by the cell.
b. To control the entry of cholesterol into the cell via LDL
c. To control the entry of cholesterol precursors into the cell via LDL
d. To control the release of cholesterol from the cell via LDL.

A

b

26
Q

How does intracellular cholesterol regulate the number of LDL receptors on the cell surface?

Cholesterol affects endocytosis and exocytosis of the LDL receptor
Cholesterol in the plasma membrane inhibits the LDL receptor activity
Cholesterol levels control SREBP Cleavage Activating Protein (SCAP) activity
Cholesterol uses LDL receptors in the cell as a storage device.

A

c

27
Q

Familial hypercholesterolemia is a disease that involves defective LDL cellular receptors, such that the cells do not take up LDL at a normal rate. Which of the following would NOT be a result of this disease?

increased blood LDL levels
increased synthesis of cholesterol in cells
fatty deposits in coronary arteries and elsewhere
increased endocytosis of LDLs.

A

d

28
Q

A patient has an enzyme deficiency that prevents the conversion of the peroxyl group at C-15 on Prostaglandin G to an –OH group during the metabolism of this molecule. How will this affect the biologic function of the PGG molecule?

A. The peroxyl-bearing molecule will have a much longer half-life and be far more potent.
B. The peroxyl-bearing molecule will behave identically to the - OH bearing molecule.
C. The peroxyl-bearing molecule will be completely inactive

A

c

29
Q

A patient has an enzyme deficiency that completely blocks production of ALL PG, TX and LT molecules. Which of the following enzyme deficiencies can account for this observation?

a. phospholipase A2
b. cyclo-oxygenase
c. lipoxygenase
d. Cytochrome P450

A

a