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

Unit 2: Protein and Nucleic Acid Biochemistry Flashcards

(74 cards)

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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
Name activators (2) and inhibitors (2) of Glu dehydrogenase
ADP, GDP; ATP, GTP
26
Name amino acids that can undergo ketogenic or glucogenic metabolism (4)
Isoleucine, tryptophan, phenylalanine, tyrosine
27
Name the enzyme complex responsible for elevated alpha-keto acids in Maple Syrup Urine Disease (MSUD)
Branched-Chain alpha-keto-acid dehydrogenase complex
28
Name the inputs for the urea cycle (4).
Ammonium, ATP, aspartate, bicarbonate
29
Name the outputs of the urea cycle (4).
P, ADP/AMP, fumarate, urea
30
Name the primary ketogenic amino acids. (2)
Leucine, lysine
31
Name two special amino acids that are important to collagen synthesis.
Hydroxyproline, hydroxylysine
32
Name two unique enzymes involved in branched amino acid metabolism.
Branched-chain aminotransferase (deamination); Branched-chain alpha-keto-acid dehydrogenase complex (decarboxylation)
33
Phenylalanine > _______ (_______) > ________ (_______) >>> catecholamines and melanin
Tyrosine; Phenylalanine hydroxylase; DOPA; Tyrosine hydroxylase
34
Urea Cycle: Arginine > _______ (________)
Urea, Ornithine; Arginase
35
Urea Cycle: Argininosuccinate > _______ (_______)
Arginine; Argininosuccinate lyase
36
Urea Cycle: Citrulline + _______ > ________ (________)
Aspartate; Argininosuccinate; argininosuccinate synthase
37
Urea Cycle: Ornithine > ________ (_________)
citrulline; carbamoyl phosphate synthetase 1
38
Vitamin B6 is a precursor for _______, which holds and transfers amino groups on aminotransferases
pyridoxal phosphate (PLP)
39
Vitamin K is an important cofactor for ________ (enzyme)
glutamyl carboxylase
40
What byproducts are produced in PKU? Which has a distinctive smell?
Phenylacetate, phenyllactate, phenylpyruvate; phenyllactate
41
What carbon donor methylates cytosine residues in DNA and methylates norepinephrine to epinephrine?
S-adenosylmethionine (SAM)
42
What enzyme deficiency is responsible for homocysteinuria?
cystathione-beta-synthase (CBS)
43
What enzyme is involved in metabolism of tryptophan? What cofactor is required?
Tryptophan hydroxylase; tetrahydrobiopterin/BH4
44
What is the biochemical defect that results from vitamin C deficiency in scurvy?
Inability to synthesize hydroxyproline and hydroxylysine
45
What is the biochemical defect that results in phenylketonuria?
Phenyalanine Hydroxylase Deficiency
46
What is the biochemical etiology of cysteinuria that can cause renal stones?
Defective cysteine transporter
47
What is the first base produced in de novo purine synthesis?
Inositol monophosphate (IMP)
48
What is the first base produced in de novo pyrimidine synthesis?
Uracil monophosphate (UMP)
49
What is the inheritance pattern for Lesch-Nyhan Disease?
Autosomal Recessive
50
What is the key regulated enzyme in de novo purine synthesis?
Glutamine Phosphoribosyl pyrophosphate Amidotransferase
51
What is the key regulated enzyme in de novo pyrimidine synthesis?
carbamoyl phosphate synthetase II
52
What is the secondary key regulated enzyme in de novo purine synthesis?
PRPP synthase
53
Which amino acid is a precursor for serotonine, melatonin, and niacin?
Tryptophan
54
Which amino acid is used to produce SAM?
methionine
55
Which amino acid is used to synthesize T4/thyroxine?
Tyrosine
56
Which enzyme is deficient in Lesch-Nyhan Syndrome? What is the effect on de novo purine synthesis?
HGPRT (Hypoxanthine-Guanine Phosphoribosyltransferase); increased de novo synthesis
57
Which enzyme is mutated in Severe Common Immunodeficiency (SCID)? What builds up as a consequence?
Adenosine Deaminase; dATP
58
Which enzyme? GSH > GSSG
Glutathione peroxidase
59
Which enzyme? GSSG > GSH
Glutathione reductase
60
Which important cofactor is responsible for holding and transfering groups on aminotransferases?
pyridoxal phosphate (PLP)
61
Which metal is required as a cofactor in heme metabolism (delta-aminolevulinate dehydratase)?
Zinc
62
Which molecule is recycled in the urea cycle?
Ornithine
63
Which molecule is used in deoxyribonucleotide synthesis? What substrates must be used?
Ribonucleotide Reductase; NDPs
64
Which molecules serve as nitrogen donors to urea in the urea cycle?
Aspartate, ammonium
65
Which pathologic pattern? Swollen gums, bruising, anemia; collagen defects
Scurvy
66
Which pathologic pattern? Uric acid crystal deposition, self-mutilating behavior, severe mental disorders
Lesch-Nyhan Syndrome
67
Which peptide maitnains iron in the ferrous state?
Glutathione (GSH)
68
Which protein degradation mechanism is ATP-dependent?
Ubiquitination-Proteasome
69
Which protein degradation mechanism is ATP-independent?
Lysosomal Degradation
70
Which special amino acid is important for targeting proteins to membranes by calcium chelations?
Gamma-carboxyglutamate
71
Which special amino acid is important in collagen hydrogen bonding and strength?
Hydroxyproline
72
Which special amino acid is important in collagen interchain crosslinks?
Hydroxylysine
73
Which special amino acid is important in prothrombin?
Gamma-carboxyglutamate
74
Which urea cycle reaction occurs in the mitochondria? Which enzyme catalyzes this reaction?
Conversion of ornithine to citrulline by carbamoyl phosphate synthetase 1

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