FEU NRMF PROTEIN METABOLISM 2014

D 1. True regarding glycine:

D A. It is a component of glutathione and creatinine.
D B. Oxidative deamination converts it to pyruvate
C. It is excreted as hippuric acid if it reacts with benzoic acid.
2. True regarding alanine:
A. It is the most active amino acid, metabolically speaking.
B. Oxidative deamination converts it to pyruvate.
C. It can be synthesized from the catabolism of threonine.
3. True regarding glutamic acid:
A. It is a component of glutathione and creatinine.
B. It is both a glycogenic and a ketogenic.
C. It is the source of the natural tranquilizer of the brain.
4. True regarding histidine:
A. Its catabolism leads to the synthesis of glutamic
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acid.
B. Histidine load test can be used to diagnose folic acid deficiency.
C. Histamine is derived from it through decarboxylation.
5. True regarding the inborn errors of protein metabolism:
A. PKU is characterized by the accumulation in the urine of the keto acids derived from Phe and
Tyr.
B. Blue-diaper syndrome is manifested by hypercalcemia and indicanuria.
C. Histidinemia can lead to ammonia intoxication.
6. True regarding the urea cycle:
A. It can be linked to the Krebs cycle through argininosuccinate.
B. It can lead to the synthesis of a semi-essential amino acid.
C. Deficiency of the ornithine transporter gene stops the urea cycle after the first reaction.
7. Important substances derived from the amino acids:
A. Nitric oxide is derived from arginine through oxidative deamination.
B. Lysine and methionine are components of carnitine.
C. Taurine, which can be converted to a bile salt, is derived from alanine.
8. Non-essential amino acid that can be synthesized from other non-essential amino acids:
A. Serine
B. Proline
C. Glutamic acid
9. True regarding the aromatic amino acids:
A. They are all dispensable amino acids.
B. They are mainly catabolized in the liver.
C. PKU leads to their accumulation in the brain, causing mental retardation.
10. True regarding transamination:
A. The most active amino group acceptor is alpha-ketoglutarate.
B. It requires vitamin B6 as the amino group carrier.
C. Transamination leads to the synthesis of non-essential amino acids.
11. Sources of amino acids in the amino acid pool of the body:
A. Breakdown of tissue proteins during protein turnover
B. Synthesis of indespensible amino acids in the liver
C. Dietary intake of proteins

12. True regarding the gamma-glutamyl cycle:

A. It requires 4 ATPs per amino acid that is absorbed.
B. It requires the presence of reduced glutathione.
C. It requires that presence of the membrane-bound enzyme gamma-glutamyl dehydrogenase.

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 13. What is/are the circumstance/s that lead/s to coma in severe liver diseases?
A. The stopping of the urea cycle
B. The depletion of glutamic acid
C. The depletion of alpha-ketoglutarate
D 14. True regarding the HHH syndrome:
A. This is due to the mutation of the ornithine transporter gene.
B. Hyperornithinemia results because ornithine canot enter the mitochondria.
C. Homocitrullinuria results because ornithine is channeled towards citrulline formation
15. Enzymes that are involved in the detoxification of ammonia:
A. Glutamate dehydrogenase
B. Aminotransferases
C. Glutaminase
16. In the treatment of ammonia intoxication:
A. Levulose is given to preven absorption of ammonia in the gut.
B. Antibiotics are given to prevent synthesis of ammonia by the bacterial flora.
C. Compounds that bind amino acids are given to excrete amino acids in the urine.
D 17. True regarding phenylalanine and tyrosine:
A. They are intercovertible through oxidation.
B. They are classified to be both glucogenic and ketogenic.
C. Their major catabolic pathway proceeds through the formation of catecholamines and melanin.
18. True regarding the different inborn errors of metabolism:
A. Eye and skin lesions are prominent manifestations of type I tyrosinemia.
B. Isovaleric academia is manifested by mental retardation.
C. The triad of alkaptonuria includes black urine, arthritis and ochronosis.
D 19. The ammonias are detoxified per turn of the urea cycle. They enter the cycle in the following steps:
A. Synthesis of citrulline
B. Synthesis of carbamoyl phosphate
C. Synthesis of fumarate and arginine
20. Amino acids that enter the Krebs cycle via alpha-ketoglutarate:
A. Proline
B. Histidine
C. Arginine
21. Enzyme/s that can be used to interconvert amino acids:
A. Glutamate dehydrogenase
B. Serine hydroxymethyltransferase
C. Phe hydroxylase
22. Pathway/s where glycine is needed:
A. Heme synthesis
B. Creatine synthesis
C. Synthesis of bile salts
23. Inborn error/s of protein metabolism that is/are manifested by kidney stone formation:
A. Carcinoid syndrome
B. Cystinuria
C. Blue diaper syndrome

24. Inborn error/s of protein metabolism that is/are manifested by mental retardation:
A. Maple syrup urine disease
B. Hartnup disease
C. Hyperlysinemia
25. SAM acts as a methyl donor in the following reactions:
A. Synthesis of choline
B. Synthesis of homocysteine
C. Synthesis of melanin

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ANSWER KEY!
1. D - 16. E
2. B 17. D -
3. C 18. C
4. E 19. D
5. B 20. E
6. D - 21. B
7. B 22. E
8. E 23. D – B and C only
9. B 24. D – A and C only
10. E 25. A
11. D -
12. B
13. E
14. D -
15. E

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