EXAM 1 Flashcards

1
Q

Gly

A

Glycine

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2
Q

Ala

A

Alanine

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3
Q

Val

A

Valine

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4
Q

Leu

A

Leucine

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5
Q

Ile

A

Isoleucine

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6
Q

Phe

A

Phenylalanine

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7
Q

Trp

A

Tryptophan

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8
Q

Met

A

Methionine

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9
Q

Pro

A

Proline

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10
Q

Asp

A

Aspartate

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11
Q

Glu

A

Glutamate

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12
Q

Ser

A

Serine

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13
Q

Thr

A

Threonine

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14
Q

Asn

A

Asparagine

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15
Q

Gln

A

Glutamine

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16
Q

Cys

A

Cysteine

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17
Q

His

A

Histidine

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18
Q

Lys

A

Lysine

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19
Q

Arg

A

Arginine

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20
Q

Acidic aminos acids are ?

A

Aspartate (asp)

Glutamate (glu)

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21
Q

Basic amino acids are?

A

Histidine (his)
Lysine (lys)
Arginine (arg)

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22
Q

Polar amino acids are ?

A
Serine/ ser 
Threonine/ thr 
Tyrosine/ tyr
Asparagine/ asn 
Glutamine / gln 
Cysteine/ cys
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23
Q

Nonpolar aminos acids are?

A
Glycine/ gly 
Alanine/ ala 
valine / Val 
Leucine / leu 
Isoleucine / ile 
Phenylalanine / phe 
Tryptophan / trp 
Methionine / met 
Proline / pro
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24
Q

T/F only L form of amino acids are used in human metabolism

A

T

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25
Q

Racemic mixture is?

A

Means there is a 1 to 1 ratio L and D isomers of the substance

Eg. racemic mixture 100mg of glycine
50 mg of L 50 mg of D (D not useful)

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26
Q

Essential amino acids?

A

Phenylalanine
Valine
Tryptophan

Threonine
Isoleucine
Methionine

Histidine
Lysine
Leucine

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27
Q

Conditionally essential AAs?

A

Arginine

Glutamine

Glycine

Taurine

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28
Q

Non essential amino acids are?

A
Alanine 
Asparagine 
Aspartic acid 
Cysteine 
Glutamic acid
Proline
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29
Q

When should we test amino acids?

A
Neonatal screening
Poor digestion 
High cholesterol 
Alzheimer’s 
Chronic fatigue 
Aging 
Cancer 
Pregnancy
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30
Q

2 exclusively ketogenic amino acids

A

Lysine

Leucine

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31
Q

AA that is principle supplier of nitrogen in the body

A

Glutamine

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32
Q

Protein turnover leads to hydrolysis and resynthesis of Xg of protein a day

A

300 to 400

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33
Q

2 major enzyme systems responsible for protein degradation

A
  1. Ubiquitin-proteasome system cytosol ATP dependent

2. Lysomal enzymes ATP independent

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34
Q

Typical grams of protein consumed daily in American diet

A

70-100

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35
Q

T/F only free amino acids are found in the portal vein after a meal

A

T

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36
Q

T/F the concentration of free amino acids is lower outside the cell than inside ?

A

False

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37
Q

T/F cystinuria is the most common genetic error of amino acid metabolism

A

t

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38
Q

2 sources of urea nitrogen

A

Ammonia

Aspartate

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39
Q

Allosteric regulator of the urea cycle?

A

NAG/N-Acetyl-glutamate

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40
Q

Where are 2 nitrogen’s provided from to form urea?

A
  1. Free ammonia (NH3)

2. Aspartate (ultimately from glutamate)

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41
Q

T/F hyperammonemia is toxic to the CNS?

A

T

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42
Q

Glucogenic amino acids are amino acids whose catabolism yields X or Y?

A
X = pyruvate 
Y = other intermediate of Citric Acid Cycle 

*** THEN PRODUCE GLUCOSE VIA GLUCONEOGENESIS

43
Q

T/F Glucogenic amino acids produce glucose via glycolysis?

A

F; thru gluconeogenesis

44
Q

Branch chain amino acids are?

A

Valine
Leucine
Isoleucine

45
Q

BCAAs are good for what type of activity?

A

Strenuous muscle activity; create a flow of alanine and glutamine from skeletal muscles

Those AAs then used for gluconeogenesis

46
Q

T/F Valine and Isoleucine used to creat succinate? Why is that important?

A

T

Succinate is TCA intermediate (create energy)

47
Q

Elevations in BAIB present with what conditions (x3)?

A
  1. B6 deficiency
  2. Leukemia
  3. BAIB pyruvate transaminase deficiency
48
Q

Amino acids involved in heme, nucleotide, and cell memebranes

A

Glycine

Alanine

49
Q

Aminotranferase/transaminase enzymes always requires what b vitamin cofactor?

A

B6

50
Q

Function of phosphotidalcholine/lecithin

A

EMULSIFIES FAT

51
Q

What is the most toxic of amino acids?

A

Methionine

52
Q

What is the PRIMARY function of amino acids?

A

Protein synthesis

53
Q

Responsibilities of mTOR? (3)

A

Mitochondrial retraction (when mitochondria shrink for survival reasons during INCREASED OXIDATIVE STRESS)

Deficiency in SULFUR AMINO ACIDS and IRON (mTOR ^ when Sulfur/Iron down)

Reversing Chronic Hypometabolic Compensatory States

54
Q

When to use amino acid testing? (9)

A
  1. NEONATAL SCREENING
  2. Poor digestion
  3. High cholesterol
  4. Alzheimer’s
  5. Chronic fatigue
  6. Chronically ill
  7. Aging
  8. Cancer
  9. Pregnancy
55
Q

Arginine requires what mineral as a cofactor?

A

MANGANESE

56
Q

What is the most dominant free amino acid in fasting plasma

A

GLUTAMINE (gln)

57
Q

Signs of ARGININE deficiency

A
  • HYPERAMMONIA (confusion, disorientation)
  • Rash
  • Hair loss
  • Poor wound healing
  • FATTY LIVER
58
Q

Function of ADMA

A

ADMA INHIBITS the synthesis of Nitric Oxide

Low NO = Endothelial dysfunction

59
Q

What condition is associated with a SNP to the ORNT1 gene?

A

“HHH”

Hyperammonemia
Hyperargininemia
Hyperornithinemia

60
Q

T/F Treatment of HHH requires a low protein diet, B6, and Mg?

A

T

61
Q

What reaction does L-Asparaginase catalyze?

A

Asparagine —> Aspartate + Ammonia

62
Q

What conditions should you AVOID supplementing with GLUTAMINE (GLN)? (3)

A

Cancer
Liver disease
Chron’s

63
Q

Is GABA inhibitory or excitatory?

A

INHIBITORY

64
Q

Large neutral amino acids are?

A
Met 
Phe
Tyr
Trp 
Leu 
Ile 
Val
65
Q

Methionine synthase requires which cofactors? (3)

A

B9
B12
Betaine

66
Q

What nutrients are needed for the Branched Chain Ketoacid Dehydrogenase Complex? (5)

A
  1. B1
  2. B2
  3. B3
  4. B5
  5. Lipoic acid
67
Q

What is the cofactor for BCAA Transaminase (hint: “when in doubt”)

A

B6

68
Q

Uses of threonine (3 main)

A
  1. Protein synthesis
  2. MS (manage spasticity)
  3. Glutathione generation
69
Q

What is the cofactor for histidine decarboxylase? (hint: when in doubt)

A

B6

70
Q

Symptoms of prenatal protein deficiency (hint: thrift)

A
  1. Increased eating
  2. Increased preference for calorie rich foods
  3. Increased IR, hypertension, adiposity, glucose intolerance
  4. Activation of THRIFT GENES leading to Met Syn
71
Q

How to perihepatic portal cells respond to increased local acidity? (Hint: it transport Gln through SN1-SN2 transporter to create what 2 THINGS?)

A

Ammonia?

HC03

72
Q

Elevations in BAIB may be present in which condition?

A

Leukemia

73
Q

Contraindications of Trp? (3 conditions you should avoid supplementation?)

A
  1. SSRIs
  2. Pregnancy
  3. Asthma
74
Q

What nutrients are needed for the creation of hydroxyproline and hydroxylysine?

A

Proline
Lysine
VITAMIN C
Dietary collagen

75
Q

What does Methionine adenosyltransferase enzyme catalyze?

A

The formation of SAMe

76
Q

Tryptophan metabolism requires what 4 cofactors?

A
  1. BH4
  2. Fe
  3. B6
  4. B3
77
Q

Know the difference between histamine being degraded in

  1. The liver
  2. Via extra hepatic-pathways

Enzymes?
Cofactors?

A
  1. Histamine —> Imidazole acetaldehyde
ENZYME = DAO 
COFACTOR = Copper, B2
  1. Histamine —> SAH + N-Methylhistidine

ENZYME: Histamine N-methyltransferase
COFACTOR = SAMe

78
Q

Enzyme that converts Histidine to HistaMINE

A

Histidine decarboxylase

79
Q

Histamine degradation: LIVER (enzyme/cofactor)

A

LIVER
E: DAO
C: Copper + B2/FAD

80
Q

Histamine depredations: EXTRA HEPATIC

A

E: Histamine N-methyltransferase
C: SAMe

81
Q

What amino acid should you NEVER supplement with?

A

Glu/Glutamate

82
Q

T/F serine is a precursor for the synthesis of phospholipids (cell membranes)

A

T

83
Q

High ethanolamine may reflect poor X status? (Hint: methylation)

A

SAMe

84
Q

Amino acids involved in BONE/COLLAGEN

A

PROLINE

85
Q

P AA that creates “bend” in the protein

A

Proline

86
Q

Collagen metabolism is inhibited by x & Y?

A

LACTATE

CADMIUM

87
Q

Are amino acids elevated at night or in the morning?

A

MORNING

88
Q

Common amino acidopathies

A

PKU
Maple syrup urine disease
Homocystenuria
Cirtulle

89
Q

High cholesterol associated with low levels of what 2 AAs?

A

Arginine (arg)

Glycine (gly)

90
Q

If there is a build up of orotate, what enzyme is implicated?

A

Carbomoyl phosphate synthetase (CPS)

91
Q

Hyperammonemia is most common clinical sign of which enzyme deficiency involved in the urea cycle ? (Hint: O)

A

OTC (ornithine transcarbomoylase)

92
Q

X is required for BCAAs to be incorporated into sketetal muscle? (Hint: sugar)

A

Insulin-like growth factor 1 (IGF-1)

93
Q

Maple Syrup Urine disease associated with genetic mutation of which enzyme ? (What would be elevated in urine)?

A

BCKDC

Branched Chain KETOACIDS (not amino)

94
Q

What nutrients may be deficient when AANB is elevated?

A

Thr

95
Q

T/F In the CNS, histamine can be used as a neurotransmitter? (Critical function eg. Aroused)

A

T

Control over arousal/sleep

96
Q

Individuals with Down syndrome have what AA deficiency? (Eg. L)

A

Lysine

97
Q

PKU means there is a problem converting Phe to ??

A

Tyr

98
Q

T/F Indican is a marker of bacterial metabolism of Trp?

A

T

99
Q

NMDA v GABA (neuro inhib or excitatory?)

A

NMDA = excitatory

GABA = inhibitory

100
Q

Is oxidative stress high or low is homocysteine goes down trans-sulfuration pathway?

A

HIGH

101
Q

Methionine is best absorbed with a little bit of ?

A

CARB

102
Q

Non animal source of SAMe?

A

Sunflower seeds

103
Q

Low Hcy would impair production of?

A

Glutathione

104
Q

High homocysteine effects which 3 body systems?

A

CNS
Musculoskeletal
Cardiovascular