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Metabolism and Nutrition > Unit 2: Protein and Nucleic Acid Biochemistry > Flashcards

Unit 2: Protein and Nucleic Acid Biochemistry Flashcards

1
Q

______ > _______ (cystathion-gamma-lyase)

A

cystathione; cysteine

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2
Q

______ is an allosteric activator of N-acetylglutamate synthase.

A

Arginine

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3
Q

______ is an amino acid used to carry nitrogen to the liver from peripheral tissues.

A

Glutamine

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4
Q

_______ > ________ (cystathione-beta-synthase)

A

homocysteine; cystathione

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5
Q

_______ is an allosteric activator of carbamoyl phosphate synthetase 1.

A

N-acetylglutamate

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6
Q

________ > _________ (Glu dehydrogenase)

A

Glutamate; alpha-ketoglutarate, ammonia

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7
Q

_____: inherited defects in heme synthesis

A

porphyrias

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8
Q

dTTP synthesis: UDP > ____ > ____ > ___> dTTP

A

dUDP; dUMP; dTMP

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9
Q

Excess enzymatic acitivty of _______ may result in overproduction of uric acid and subsequent gout.

A

Xanthine oxidase

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10
Q

Glutathione ______ may be involved in detoxification, drug resistance, and tumors

A

Glutathione-S-transferase

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11
Q

Glutathione ______ mutations may lead to heme problems

A

Glutathione reductase

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12
Q

Glutathione ________ mutations may lead to higher cancer risk.

A

Glutathione peroxidase

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13
Q

Hyperhomocyteinemia can result from deficiencies of ______ (3).

A

B6, B12, folate

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14
Q

In _____ degradation, the base is opened and used in other pathways while in ______ degradation, uric acid is produced and excreted in the urine

A

Pyrimidine; Purine

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15
Q

In _____ synthesis, the base is pre-synthesized and then attached to the sugar

A

Pyrimidine

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16
Q

In _____ synthesis, the base is synthesized directly on the sugar.

A

Purine

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17
Q

In Lesch-Nyhan disease, levels of _____ are increased, while levels of _____ are decreased.

A

PRPP; GMP, IMP

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18
Q

In nitrogen metabolism, arginine may be converted to _________. (3)

A

ornithine, citrulline (NO), creatine phosphate

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19
Q

Inability to synthesize new dNTPs in SCID results from inhibition of _______.

A

Ribonucleotide Reductase

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20
Q

Muscle converts pyruvate to _______ to transport nitrogen to the liver.

A

alanine

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21
Q

Name 3 enzymes that require BH4 as a cofactor.

A

Tyrosine hydroxylase, tryptophan hydroxylase, phenylalanine hydroxylase

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22
Q

Name 5 sources of atoms in purine synthesis

A

Glycine, glutamine, aspartate, THF, carbon dioxide

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23
Q

Name a potential treatment for cysteinuria.

A

Acetazolamide

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24
Q

Name a potential treatment for homocysteinuria.

A

Vitamin B6

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25
Q

Name activators (2) and inhibitors (2) of Glu dehydrogenase

A

ADP, GDP; ATP, GTP

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26
Q

Name amino acids that can undergo ketogenic or glucogenic metabolism (4)

A

Isoleucine, tryptophan, phenylalanine, tyrosine

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27
Q

Name the enzyme complex responsible for elevated alpha-keto acids in Maple Syrup Urine Disease (MSUD)

A

Branched-Chain alpha-keto-acid dehydrogenase complex

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28
Q

Name the inputs for the urea cycle (4).

A

Ammonium, ATP, aspartate, bicarbonate

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29
Q

Name the outputs of the urea cycle (4).

A

P, ADP/AMP, fumarate, urea

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30
Q

Name the primary ketogenic amino acids. (2)

A

Leucine, lysine

31
Q

Name two special amino acids that are important to collagen synthesis.

A

Hydroxyproline, hydroxylysine

32
Q

Name two unique enzymes involved in branched amino acid metabolism.

A

Branched-chain aminotransferase (deamination); Branched-chain alpha-keto-acid dehydrogenase complex (decarboxylation)

33
Q

Phenylalanine > _______ (_______) > ________ (_______)&raquo_space;> catecholamines and melanin

A

Tyrosine; Phenylalanine hydroxylase; DOPA; Tyrosine hydroxylase

34
Q

Urea Cycle: Arginine > _______ (________)

A

Urea, Ornithine; Arginase

35
Q

Urea Cycle: Argininosuccinate > _______ (_______)

A

Arginine; Argininosuccinate lyase

36
Q

Urea Cycle: Citrulline + _______ > ________ (________)

A

Aspartate; Argininosuccinate; argininosuccinate synthase

37
Q

Urea Cycle: Ornithine > ________ (_________)

A

citrulline; carbamoyl phosphate synthetase 1

38
Q

Vitamin B6 is a precursor for _______, which holds and transfers amino groups on aminotransferases

A

pyridoxal phosphate (PLP)

39
Q

Vitamin K is an important cofactor for ________ (enzyme)

A

glutamyl carboxylase

40
Q

What byproducts are produced in PKU? Which has a distinctive smell?

A

Phenylacetate, phenyllactate, phenylpyruvate; phenyllactate

41
Q

What carbon donor methylates cytosine residues in DNA and methylates norepinephrine to epinephrine?

A

S-adenosylmethionine (SAM)

42
Q

What enzyme deficiency is responsible for homocysteinuria?

A

cystathione-beta-synthase (CBS)

43
Q

What enzyme is involved in metabolism of tryptophan? What cofactor is required?

A

Tryptophan hydroxylase; tetrahydrobiopterin/BH4

44
Q

What is the biochemical defect that results from vitamin C deficiency in scurvy?

A

Inability to synthesize hydroxyproline and hydroxylysine

45
Q

What is the biochemical defect that results in phenylketonuria?

A

Phenyalanine Hydroxylase Deficiency

46
Q

What is the biochemical etiology of cysteinuria that can cause renal stones?

A

Defective cysteine transporter

47
Q

What is the first base produced in de novo purine synthesis?

A

Inositol monophosphate (IMP)

48
Q

What is the first base produced in de novo pyrimidine synthesis?

A

Uracil monophosphate (UMP)

49
Q

What is the inheritance pattern for Lesch-Nyhan Disease?

A

Autosomal Recessive

50
Q

What is the key regulated enzyme in de novo purine synthesis?

A

Glutamine Phosphoribosyl pyrophosphate Amidotransferase

51
Q

What is the key regulated enzyme in de novo pyrimidine synthesis?

A

carbamoyl phosphate synthetase II

52
Q

What is the secondary key regulated enzyme in de novo purine synthesis?

A

PRPP synthase

53
Q

Which amino acid is a precursor for serotonine, melatonin, and niacin?

A

Tryptophan

54
Q

Which amino acid is used to produce SAM?

A

methionine

55
Q

Which amino acid is used to synthesize T4/thyroxine?

A

Tyrosine

56
Q

Which enzyme is deficient in Lesch-Nyhan Syndrome? What is the effect on de novo purine synthesis?

A

HGPRT (Hypoxanthine-Guanine Phosphoribosyltransferase); increased de novo synthesis

57
Q

Which enzyme is mutated in Severe Common Immunodeficiency (SCID)? What builds up as a consequence?

A

Adenosine Deaminase; dATP

58
Q

Which enzyme? GSH > GSSG

A

Glutathione peroxidase

59
Q

Which enzyme? GSSG > GSH

A

Glutathione reductase

60
Q

Which important cofactor is responsible for holding and transfering groups on aminotransferases?

A

pyridoxal phosphate (PLP)

61
Q

Which metal is required as a cofactor in heme metabolism (delta-aminolevulinate dehydratase)?

A

Zinc

62
Q

Which molecule is recycled in the urea cycle?

A

Ornithine

63
Q

Which molecule is used in deoxyribonucleotide synthesis? What substrates must be used?

A

Ribonucleotide Reductase; NDPs

64
Q

Which molecules serve as nitrogen donors to urea in the urea cycle?

A

Aspartate, ammonium

65
Q

Which pathologic pattern? Swollen gums, bruising, anemia; collagen defects

A

Scurvy

66
Q

Which pathologic pattern? Uric acid crystal deposition, self-mutilating behavior, severe mental disorders

A

Lesch-Nyhan Syndrome

67
Q

Which peptide maitnains iron in the ferrous state?

A

Glutathione (GSH)

68
Q

Which protein degradation mechanism is ATP-dependent?

A

Ubiquitination-Proteasome

69
Q

Which protein degradation mechanism is ATP-independent?

A

Lysosomal Degradation

70
Q

Which special amino acid is important for targeting proteins to membranes by calcium chelations?

A

Gamma-carboxyglutamate

71
Q

Which special amino acid is important in collagen hydrogen bonding and strength?

A

Hydroxyproline

72
Q

Which special amino acid is important in collagen interchain crosslinks?

A

Hydroxylysine

73
Q

Which special amino acid is important in prothrombin?

A

Gamma-carboxyglutamate

74
Q

Which urea cycle reaction occurs in the mitochondria? Which enzyme catalyzes this reaction?

A

Conversion of ornithine to citrulline by carbamoyl phosphate synthetase 1

Metabolism and Nutrition (10 decks)

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