14 - Amino Acid Metabolism

Question 1

What is the difference between essential and non-essential amino acids? Which amino acids are which?

Answer 1

Conditionally essential amino acids are only essential when there is a defect in conversion of the essential amino acid to the conditionally essential amino acid

Question 2

What is the difference between glucogenic and ketogenic amino acids?

Answer 2

Question 3

How are amino acids digested after ingestion?

Answer 3

Question 4

What is different in amino acid metabolism compared to lipid/carbohydrate metabolism?

Answer 4

Question 5

What is the waste product of amino acid metabolism and how is it disposed of? What is the concept of nitrogen balance?

Answer 5

Question 6

Answer 6

A

Question 6

Answer 6

C

Question 7

Give an overview of amino acid and nitrogen metabolism.

Answer 7

Question 8

How is nitrogen removed from amino acids so their carbon backbone can be used in the TCA cycle?

Answer 8

Question 9

Describe Transamination. Why is it important in amino acid metabolism? Where is its products used and what are they used for?

Answer 9

Question 10

What is the clinical relevance of aminotransferases?

Answer 10

Question 11

Explain oxidative deamination.

Answer 11

Question 12

Answer 12

D (C is also partially incorrect as ammonia is never truly free)

Question 13

What is urea? How is it synthesized in the body? Why is it important? Describe the Urea cycle. How is this cycle controlled?

Question 14

What are the hallmarks of urea cycle disorders?

Answer 14

Question 15

Answer 15

B and E

Question 16

What are the most common diseases associated with amino acid metabolism?

Answer 16

Question 17

Explain Phenyketonuria. What are its symptoms? How is it treated?

Answer 17

Question 18

Answer 18

B

Question 19

Explain Alkaptonuria. What are its symptoms? How is it treated?

Answer 19

Question 20

Explain Albinism. What are its symptoms? How is it treated?

Answer 20

Question 21

Explain Homocystinuria. What are its symptoms? How is it treated?

Answer 21

Question 22

Explain Maple syrup urine disease (MSUD). What are its symptoms? How is it treated?

Answer 22

Question 23

Answer 23

C

Question 24

Give an overview of the defects in the amino acid transporters.

Answer 24

Question 25

Explain Bue Diaper syndrome. What are its symptoms? How is it treated?

Answer 25

Question 26

Explain cystinuria. What are its symptoms? How is it treated?

Answer 26

Question 27

What are the amino acid metabolism disorders? What are their symptoms? How are they treated?

Answer 27

Question 28

Answer 28

C

Question 29

Answer 29

D

Question 30

Answer 30

G

Question 31

Answer 31

B

Question 32

Answer 32

B

Question 33

Answer 33