Urea Cycle Flashcards

1
Q

Urea Cycle Big Picture

A
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2
Q

3 Transaminations

A
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3
Q

Vitamin B6 and AA Metabolism

A
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4
Q

What other enzyme requires vitamin b6?

Glucokinase

Glycogen Phosphorylase

Pyruvate Dehydrogenase

G6P dehaydrogenas

Fatty Ayl CoA Synthetase

A

Glycogen Phosphorylase

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5
Q

Muscle Degredation of Protein Will Occur under which of the following Conditions?

After a high fat low carb meal

During prolonged fasting

After a high carb low protein meal

During shorter anaerobic exercise

A

After a high fat low carb meal — This might be accepted much further downstream.

During prolonged fasting

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6
Q

Muscle Degredation of Amino Acids

A
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7
Q

Ammonia Metabolism in Liver

A
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8
Q

Enzymes involved in Nitrogen Metabolism

A
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9
Q

Flow of Nitrogen

A
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10
Q

Glutamine Synthetase

A

Glutamine Synthetas is found in the cytoplasm.

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11
Q

Glutaminase

A

So is Glutaminase (found in the cytosol).

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12
Q

Glutamate Dehydrogenase

A
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13
Q

Why is Ammonia Toxic?

A
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14
Q

Nervous System and Amino Acids

A
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15
Q

Synthesis of Carbamoyl Phosphate

A
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16
Q

Urea Cycle Pathway

A
17
Q

Compartmentation in the Urea Cycle

A
18
Q

TCA Urea Cycle Interactions

A
19
Q

Where does Ornithine Come From?

A
20
Q

Regulation of the Urea Cycle

A
21
Q

What other pathway exhibited long term adaptation?

Glycolysis

Gluconeogenesis

Fatty acid Syntehisis

Fatty Acid Oxidation

A

Fatty acid Syntehisis

22
Q

Allosteric Regulation of the Urea Cycle

A
23
Q

Pathway for Regulation of Urea Cycle

A
24
Q

N-Acetyl Glutamate Synthetase Deficiency and CPS- Deficiency

A
25
Q

Ornithine Transcarbamoylase Deficiency

A
26
Q

Continued Disorders of the Urea Cycle

A
27
Q

Arginase Defect

A
28
Q

Potential Treatment for urea cycle disorders could include which of the following?

Take aspirin

Taking essential amino acids

Taking Alpha keto acids of essential amino acids

Increasing the protein content of diet

Decreasing Carbohydrate Intake

A

Taking Alpha keto acids of essential amino acids — leads to reduced ammonia levels.

29
Q

Treatment for Urea Cycle Disorders

A
30
Q

Hypercitrullinemia Type II

A
31
Q

Nitrogen Balance

A
32
Q

Case Study

A

Ornithine Transcarbamylase deficiency

33
Q

A Healthy individualstarts a diet which had adequate calories but lacks one essential amino acid. What happens to their nitrogen balance?

Neutral Nitrogen Balance

Positive Nitrogen Balance

Negative Nitrogen Balance

A

Negative Nitrogen Balance