Test 4 questions and answers Flashcards

1
Q
  1. Indicate the direct carbon donor in the thymidylate synthase reaction?

A) N5,N10-methylene tetrahydrofolate

B) N10-formyl tetrahydrofolate

C) 7,8-dihydrofolate

D) Tetrahydrofolate

A

A) N5,N10-methylene tetrahydrofolate

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2
Q
  1. Which amino acid is required for the synthesis of -amino levulinate (ALA) in the heme synthesis pathway?

A) glutamate

B) glutamine

C) glycine

D) alanine

A

C) glycine

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3
Q
  1. What is a key regulatory enzyme in heme biosynthesis?

A) Ferrochelatase

B) δ-aminolevulinate synthase 1

C) Protoporphirynogen oxidase

D) Uroporphirynogen decarboxylase

A

B) δ-aminolevulinate synthase 1 (ALAS1)

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4
Q
  1. What are the symptoms of congenital erythropoietic porphyria?: [W]

A) photosensitivity

B) increased urinary and fecal uroporphyrin I

C) abdominal pain

D) neuropsychiatric symptoms

A

A) photosensitivity

B) increased urinary and fecal uroporphyrin I

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5
Q
  1. In the synthesis of which amino acid is the 3-phosphoglycerate dehydrogenase enzyme
    involved?:

A) Alanine

B) Methionine

C) Serine

D) Glycine

A

C) Serine

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6
Q
  1. Which of the following amino acids is not broken down to alpha-ketoglutarate?:

A) histidine

B) tryptophan

C) arginine

D) glutamine

A

B) tryptophan

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7
Q
  1. Choose amino acids that decompose to fumarate: [W]

A) Tryptophan

B) Phenylalanine

C) Tyrosine

D) Histidine

A

A) Tryptophan

B) Phenylalanine

C) Tyrosine

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8
Q
  1. Deficiency of which of the following enzymes is responsible for phenylketonuria?

A) Phenylalanyl aminotransferase

B) Tyrosine hydroxylase

C) Phenylalanyl dehydrogenase

D) Phenylalanine hydroxylase

A

D) Phenylalanine hydroxylase

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9
Q
  1. Which animals are ureotelic?
A

Panda and shark
(land vertebrates + shark)

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10
Q
  1. What are the products of arginase?
A

Ornithine and urea

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11
Q
  1. What do we do in the case of a mutation in argininosuccinate lyase?
A

We provide arginine

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12
Q
  1. How many ATP molecules are required for urea synthesis?
A

3

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13
Q
  1. The cofactor for all aminotransferases is pyridoxal phosphate (PLP)
A

Vitamin B6

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14
Q
  1. What is the intermediate of the urea cycle and Krebs cycle?
A

Fumarate

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15
Q
  1. In which disease do we administer allopurinol?
A

In gout and kidney stones

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16
Q
  1. Which amino acids are ketogenic?
A

Leucine and Lysine

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17
Q
  1. Which amino acids are branched-chain?
A

Valine, isoleucine and leucine

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18
Q
  1. In which disease is there an abundance of bilirubin
    diglucuronide?
A

Obstructive jaundice

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19
Q
  1. The end product of purine degradation is
A

Uric acid

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20
Q
  1. Which of the following compounds inhibits glutamate
    dehydrogenase?
A

GTP

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21
Q
  1. Which of the breakdown products of heme is responsible for giving urine a straw color?
A

Urobilin

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22
Q
  1. Deficiency of which enzyme is associated with phenylketonuria?
A

Phenylalanine hydroxylase

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23
Q
  1. Which of the listed amino acids does not undergo transamination?
A

Proline, hydroxyproline, threonine and lysine

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24
Q
  1. Where do the amino groups in urea come from?
A

Asparagine and ornithine

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25
Q
  1. Identify the tissues where arginine synthesis from citrulline occurs
A

Kidneys and brain

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26
Q
  1. Identify the dominant amino acids in collagen
A

Glycine and proline

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27
Q
  1. In which cellular compartment is glutamate dehydrogenase present?
A

Mitochondrial matrix

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28
Q
  1. Which of the following amino acids is not found in proteins?
A

Homohydroxy carnitine (?), ornithine and citrulline

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29
Q
  1. Which amino acid is synthesized by the Baranowski enzyme?
A

Serine

30
Q
  1. What is the final product of purine degradation in Dalmatians?
A

Uric acid

31
Q
  1. Identify the direct carbon donor in the synthesis of
    thymidylate?
A

N5N10-methylenetetrahydrofolate

32
Q
  1. Which of the amino acids is not degraded to alpha-ketoglutarate?
A

Tryptophan

33
Q
  1. Xanthylate is formed during the reactions leading to the
    formation of
A

GMP

34
Q
  1. Choose the amino acids that are broken down to fumarate
A

Tyrosine and phenylalanine

35
Q
  1. Which of the indicated amino acids is ketogenic?
A

Leucine and lysine

36
Q
  1. Which of the following amino acids is used for transporting
    amino groups from working muscles?
A

Alanine

37
Q
  1. Propose measures to alleviate the consequences of
    argininosuccinate lyase mutations
A

Reduce protein intake and administer arginine

38
Q
  1. In what form are the amino groups of amino acids excreted by
    uricotelic organisms?
A

Uric acid

39
Q
  1. Which of the listed amino acids does not undergo
    transamination?
A

Lysine, proline, threonine and hydroxyproline

40
Q
  1. Which of the listed amino acids are classified as ketogenic amino acids?
A

Lysine and leucine.

41
Q
  1. Into which of the listed compounds is phenylalanine degraded, among others?
A

Acetoacetate and fumarate

42
Q
  1. What is the name of the enzyme involved in the removal of
    α-amino groups from serine?
A

Serine dehydratase

43
Q
  1. What is the name of the enzyme that catalyzes the conversion
    of biliverdin to bilirubin?
A

Biliverdin reductase

44
Q
  1. Which of the listed amino acids are involved in the synthesis
    of the pyrimidine ring?
A

Glutamine and asparagine

45
Q
  1. Amino acids in the synthesis of the purine ring?
A

Glycine, asparagine and glutamine.

46
Q
  1. Which of the listed compounds is an allosteric activator of
    carbamoyl phosphate synthetase II (CPS II)?
A

PRPP (5-phosphoribosyl-1 pyrophosphate)

{inhibited by CTP}

47
Q
  1. What is the molecule that allows the conversion of PRPP to IMP in the salvage pathway?
A

Hypoxanthine

48
Q
  1. Which of the listed compounds serves as a transport form of
    nitrogen from muscles to the liver?
A

Glutamine and alanine

49
Q
  1. What is the compound that is the end product of purine catabolism in primates? Provide the name of the enzyme that catalyzes the reaction leading to the formation of this compound
A

Uric acid and xanthine oxidase

50
Q
  1. Complete the sentence: The urea cycle is connected to the (……) through the production of
    fumarate by the enzyme (……).
A

Krebs cycle/ citric acid cycle

argininosuccinate lyase

51
Q
  1. Besides ammonium ion and carbon dioxide, what compound is thymine degraded into?
A

Beta-aminoisobutyric acid

52
Q
  1. Porphyrins are heterocyclic compounds consisting of four
    (……) rings connected by (……) bridges
A

pyrrole

methylene

53
Q
  1. Provide the full names of two aminotransferases whose activity measurement is used in laboratory diagnostics. What is the name of the coenzyme essential for their activity?
A

Alanine aminotransferase (ALT), aspartate aminotransferase (AST) and pyridoxal phosphate

54
Q
  1. Provide the names of the compounds from which the
    synthesis of cysteine begins.
A

Homocysteine and serine

55
Q
  1. Provide the names of all the active one-carbon groups carried by tetrahydrofolate
A

Methyl, methylene, methenyl, formyl and formimino

56
Q
  1. What compound is the primary source of ammonia/ammonium ions that enter the urea cycle?
A
  • Glutamine
57
Q
  1. AMP degradation begins with the removal of the (……)
    group, leading to the formation of (……).
A

amino

IMP

58
Q
  1. In what form are the amino groups of amino acids excreted by ureotelic organisms?
A

Urea

59
Q
  1. Which of the listed amino acids is exclusively formed by the
    action of specific aminotransferases?
A

Aspartic acid/asparagine
(AST) and alanine (ALT) (aminotransferase abbreviations)

60
Q
  1. Provide the names of the products of the major pathway of
    glycine degradation in mammals
A

Carbon dioxide, ammonia and methylenetetrahydrofolate (N5,N10-methyleno-THF)

61
Q
  1. Into which of the listed compounds is valine degraded?
A

Succinyl-CoA

62
Q
  1. What is the name of the enzymatic system that catalyzes the
    degradation of heme to biliverdin?
A

Heme oxygenase

63
Q
  1. Provide the name of one of the heme proteins responsible for
    electron transport in the respiratory chain
A

Cytochrome C

64
Q
  1. What nucleotide is formed in the salvage pathway when
    hypoxanthine is attached to PRPP?
A

Inosine monophosphate
(IMP).

65
Q
  1. What is the final product of purine catabolism in non-primate
    mammals? Provide the name of the enzyme that catalyzes the reaction leading to the formation of this compound.
A

Allantoin and uricase

66
Q
  1. Serotonin is formed by the hydroxylation of (……)
    by tryptophan hydroxylase and the removal of the (……)
    group.
A

tryptophan

carboxyl

67
Q
  1. Besides ammonium ion and carbon dioxide, what compound
    is uracil degraded into?
A

Beta-alanine

68
Q
  1. The α-amino group of most amino acids is transferred to (……) by the action of glutamate transaminase,
    with (……) as a coenzyme
A

alpha-ketoglutarate

pyridoxal phosphate

69
Q
  1. Name the allosteric activators of glutamate dehydrogenase.
A

ADP - activates, (GTP - inhibits)

70
Q
  1. List, in order, the intermediates in the serine synthesis
    pathway, starting from the precursor compound:
A

3-phosphoglycerate, 3-phosphopyruvate and 3-phosphoserine.

71
Q
  1. Provide the name of the cofactor that is essential for the
    activity of carbamoyl phosphate synthetase I.
A

N-acetylglutamate (as well as Mg2+ and ATP)