EXAM 1 Flashcards
(104 cards)
1
Q
Gly
A
Glycine
2
Q
Ala
A
Alanine
3
Q
Val
A
Valine
4
Q
Leu
A
Leucine
5
Q
Ile
A
Isoleucine
6
Q
Phe
A
Phenylalanine
7
Q
Trp
A
Tryptophan
8
Q
Met
A
Methionine
9
Q
Pro
A
Proline
10
Q
Asp
A
Aspartate
11
Q
Glu
A
Glutamate
12
Q
Ser
A
Serine
13
Q
Thr
A
Threonine
14
Q
Asn
A
Asparagine
15
Q
Gln
A
Glutamine
16
Q
Cys
A
Cysteine
17
Q
His
A
Histidine
18
Q
Lys
A
Lysine
19
Q
Arg
A
Arginine
20
Q
Acidic aminos acids are ?
A
Aspartate (asp)
Glutamate (glu)
21
Q
Basic amino acids are?
A
Histidine (his)
Lysine (lys)
Arginine (arg)
22
Q
Polar amino acids are ?
A
Serine/ ser Threonine/ thr Tyrosine/ tyr Asparagine/ asn Glutamine / gln Cysteine/ cys
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
24
Q
T/F only L form of amino acids are used in human metabolism
A
T
25
Racemic mixture is?
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)
26
Essential amino acids?
Phenylalanine
Valine
Tryptophan
Threonine
Isoleucine
Methionine
Histidine
Lysine
Leucine
27
Conditionally essential AAs?
Arginine
Glutamine
Glycine
Taurine
28
Non essential amino acids are?
```
Alanine
Asparagine
Aspartic acid
Cysteine
Glutamic acid
Proline
```
29
When should we test amino acids?
```
Neonatal screening
Poor digestion
High cholesterol
Alzheimer’s
Chronic fatigue
Aging
Cancer
Pregnancy
```
30
2 exclusively ketogenic amino acids
Lysine
| Leucine
31
AA that is principle supplier of nitrogen in the body
Glutamine
32
Protein turnover leads to hydrolysis and resynthesis of Xg of protein a day
300 to 400
33
2 major enzyme systems responsible for protein degradation
1. Ubiquitin-proteasome system cytosol ATP dependent
| 2. Lysomal enzymes ATP independent
34
Typical grams of protein consumed daily in American diet
70-100
35
T/F only free amino acids are found in the portal vein after a meal
T
36
T/F the concentration of free amino acids is lower outside the cell than inside ?
False
37
T/F cystinuria is the most common genetic error of amino acid metabolism
t
38
2 sources of urea nitrogen
Ammonia
| Aspartate
39
Allosteric regulator of the urea cycle?
NAG/N-Acetyl-glutamate
40
Where are 2 nitrogen’s provided from to form urea?
1. Free ammonia (NH3)
| 2. Aspartate (ultimately from glutamate)
41
T/F hyperammonemia is toxic to the CNS?
T
42
Glucogenic amino acids are amino acids whose catabolism yields X or Y?
```
X = pyruvate
Y = other intermediate of Citric Acid Cycle
```
*** THEN PRODUCE GLUCOSE VIA GLUCONEOGENESIS
43
T/F Glucogenic amino acids produce glucose via glycolysis?
F; thru gluconeogenesis
44
Branch chain amino acids are?
Valine
Leucine
Isoleucine
45
BCAAs are good for what type of activity?
Strenuous muscle activity; create a flow of alanine and glutamine from skeletal muscles
Those AAs then used for gluconeogenesis
46
T/F Valine and Isoleucine used to creat succinate? Why is that important?
T
Succinate is TCA intermediate (create energy)
47
Elevations in BAIB present with what conditions (x3)?
1. B6 deficiency
2. Leukemia
3. BAIB pyruvate transaminase deficiency
48
Amino acids involved in heme, nucleotide, and cell memebranes
Glycine
| Alanine
49
Aminotranferase/transaminase enzymes always requires what b vitamin cofactor?
B6
50
Function of phosphotidalcholine/lecithin
EMULSIFIES FAT
51
What is the most toxic of amino acids?
Methionine
52
What is the PRIMARY function of amino acids?
Protein synthesis
53
Responsibilities of mTOR? (3)
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
When to use amino acid testing? (9)
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
Arginine requires what mineral as a cofactor?
MANGANESE
56
What is the most dominant free amino acid in fasting plasma
GLUTAMINE (gln)
57
Signs of ARGININE deficiency
- HYPERAMMONIA (confusion, disorientation)
- Rash
- Hair loss
- Poor wound healing
- FATTY LIVER
58
Function of ADMA
ADMA INHIBITS the synthesis of Nitric Oxide
Low NO = Endothelial dysfunction
59
What condition is associated with a SNP to the ORNT1 gene?
“HHH”
Hyperammonemia
Hyperargininemia
Hyperornithinemia
60
T/F Treatment of HHH requires a low protein diet, B6, and Mg?
T
61
What reaction does L-Asparaginase catalyze?
Asparagine —> Aspartate + Ammonia
62
What conditions should you AVOID supplementing with GLUTAMINE (GLN)? (3)
Cancer
Liver disease
Chron’s
63
Is GABA inhibitory or excitatory?
INHIBITORY
64
Large neutral amino acids are?
```
Met
Phe
Tyr
Trp
Leu
Ile
Val
```
65
Methionine synthase requires which cofactors? (3)
B9
B12
Betaine
66
What nutrients are needed for the Branched Chain Ketoacid Dehydrogenase Complex? (5)
1. B1
2. B2
3. B3
4. B5
5. Lipoic acid
67
What is the cofactor for BCAA Transaminase (hint: “when in doubt”)
B6
68
Uses of threonine (3 main)
1. Protein synthesis
2. MS (manage spasticity)
3. Glutathione generation
69
What is the cofactor for histidine decarboxylase? (hint: when in doubt)
B6
70
Symptoms of prenatal protein deficiency (hint: thrift)
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
How to perihepatic portal cells respond to increased local acidity? (Hint: it transport Gln through SN1-SN2 transporter to create what 2 THINGS?)
Ammonia?
HC03
72
Elevations in BAIB may be present in which condition?
Leukemia
73
Contraindications of Trp? (3 conditions you should avoid supplementation?)
1. SSRIs
2. Pregnancy
3. Asthma
74
What nutrients are needed for the creation of hydroxyproline and hydroxylysine?
Proline
Lysine
VITAMIN C
Dietary collagen
75
What does Methionine adenosyltransferase enzyme catalyze?
The formation of SAMe
76
Tryptophan metabolism requires what 4 cofactors?
1. BH4
2. Fe
3. B6
4. B3
77
Know the difference between histamine being degraded in
1. The liver
2. Via extra hepatic-pathways
Enzymes?
Cofactors?
1. Histamine —> Imidazole acetaldehyde
```
ENZYME = DAO
COFACTOR = Copper, B2
```
2. Histamine —> SAH + N-Methylhistidine
ENZYME: Histamine N-methyltransferase
COFACTOR = SAMe
78
Enzyme that converts Histidine to HistaMINE
Histidine decarboxylase
79
Histamine degradation: LIVER (enzyme/cofactor)
LIVER
E: DAO
C: Copper + B2/FAD
80
Histamine depredations: EXTRA HEPATIC
E: Histamine N-methyltransferase
C: SAMe
81
What amino acid should you NEVER supplement with?
Glu/Glutamate
82
T/F serine is a precursor for the synthesis of phospholipids (cell membranes)
T
83
High ethanolamine may reflect poor X status? (Hint: methylation)
SAMe
84
Amino acids involved in BONE/COLLAGEN
PROLINE
85
P AA that creates “bend” in the protein
Proline
86
Collagen metabolism is inhibited by x & Y?
LACTATE
CADMIUM
87
Are amino acids elevated at night or in the morning?
MORNING
88
Common amino acidopathies
PKU
Maple syrup urine disease
Homocystenuria
Cirtulle
89
High cholesterol associated with low levels of what 2 AAs?
Arginine (arg)
| Glycine (gly)
90
If there is a build up of orotate, what enzyme is implicated?
Carbomoyl phosphate synthetase (CPS)
91
Hyperammonemia is most common clinical sign of which enzyme deficiency involved in the urea cycle ? (Hint: O)
OTC (ornithine transcarbomoylase)
92
X is required for BCAAs to be incorporated into sketetal muscle? (Hint: sugar)
Insulin-like growth factor 1 (IGF-1)
93
Maple Syrup Urine disease associated with genetic mutation of which enzyme ? (What would be elevated in urine)?
BCKDC
Branched Chain KETOACIDS (not amino)
94
What nutrients may be deficient when AANB is elevated?
Thr
95
T/F In the CNS, histamine can be used as a neurotransmitter? (Critical function eg. Aroused)
T
Control over arousal/sleep
96
Individuals with Down syndrome have what AA deficiency? (Eg. L)
Lysine
97
PKU means there is a problem converting Phe to ??
Tyr
98
T/F Indican is a marker of bacterial metabolism of Trp?
T
99
NMDA v GABA (neuro inhib or excitatory?)
NMDA = excitatory
GABA = inhibitory
100
Is oxidative stress high or low is homocysteine goes down trans-sulfuration pathway?
HIGH
101
Methionine is best absorbed with a little bit of ?
CARB
102
Non animal source of SAMe?
Sunflower seeds
103
Low Hcy would impair production of?
Glutathione
104
High homocysteine effects which 3 body systems?
CNS
Musculoskeletal
Cardiovascular
