1
Q
T/F Quinolinic Acid is the link between the immune system and the brain?
A
T
2
Q
What does an elevation of HPLA indicate?
A
Cancer/tumors
3
Q
AHB (alpha-hydroxybutyrate) is directly related to the rate of hepatic X X?
A
Glutathione synthesis
4
Q
How do energy needs change in children with Down Syndrome?
A
** Decreased caloric needs (than normal baby)NEED LESS CALORIES
> 108 calories/kg
5
Q
What parts of the gut are most likely to produce microbial metabolites? (X2)
A
Terminal ileum
Ascending colon
6
Q
** What part (upper or lower) of GI tract do you tend to find AEROBIC MICROORGANISMS ? Where anaerobic?
A
Upper (mouth, stomach, beginning of SI)
ANAEROBIC = LOWER GI TRACT (small and large intestine because not exposed to O2 there)
7
Q
T/F Cresol excretion is not effected by dietary protein intake?
A
T
8
Q
** Elevations in lactate, pyruvate, and beta-hydroxybutyrate indicate abnormalities in \_\_\_\_\_\_\_\_\_\_ metabolism A. Protein B. Carb C. Fat D. Mineral
A
B. Carb
9
Q
** Elevations in suberate, adipate, and ethyl also ate indicate dysfunction of ____________ metabolism
A. Carb
B. Protein
C. Fatty acid
A
C. Fatty Acid
10
Q
**5 nutrients that are MOST IMPACTED (lowered) by intestinal dysbiosis markers
A
B1, B2, B6, Mg, Zn
11
Q
** Inhibition of CoQ10 synthesis from stating drugs will typically reveal elevations in urinary \_\_\_\_\_\_\_\_\_\_\_\_\_\_? A. Hydroxymethylproprionate B. Hydroxymethylglutarate C. Hydroxymethylbutyrate D. Hydroxymethylsuccinate
A
B.
12
Q
** Asparagine can be converted into aspartate. Aspartate is in a unique position to enter the TCA cycle through its conversion into the TCA cycle intermediate, ____________, via the enzyme AST and cofactor B6
A
Oxaloacetate
13
Q
** Low urinary citrate levels have been associated with kidney stone formation. What type of diet may INCREASE the risk of kidney stone formation?
A
HIGH protein, LOW carb
14
Q
** High MMA can inhibit the ___________ transporter
A
MALATE
15
Q
(SG) What organic acid compound can block the malate transporter?
A
MMA/ methymalonic acid
16
Q
(SG) What type of diet can INCREASE the risk of kidney stone formation?
A
HIGH protein
LOW carb
17
Q
(SG) What is the enzyme and cofactors (3) required for ISOCITRATE —> a-KG
A
E: isocitrate dehydrogenase
C: B3/NAD, Mg, Mn
18
Q
(SG) Aconitase requires which MINERAL as a cofactor?
A
Fe
19
Q
** The central energy pathways begin with digestion and assimilation of fats, carbohydrates, and proteins. Proteins require the use of _________ (5) for the conversion of keto acids into acetyl-CoA?
A
B1 B2 B3 B5 Lipoate
20
Q
** Degradation of serotonin —> 5HIAA requires what enzyme and which 2 cofactors?
A
E: MAO
C: Cu, B2
21
Q
(SG) For 5HIAA (product), know substrate, enzyme, cofactors (3)
A
S: Serotonin
E: MAO
C: Cu, B2/B3, Fe
22
Q
(SG) Know what 1. elevations of BENZOATE (4) and HIPPURATE indicate and 2. treatment
A
- DYSBIOSIS (SIBO), polyphenol bacterial catabolism, Food = ingestion of food preservatives, poor detoxification
- Glycine and B5 (if high benzoate)
23
Q
(SG) What amino acid can lead to an elevation of INDICAN due to dysbiosis?
A
Tryptophan
24
Q
(SG) What do yeast and fungi produce as a dysbiosis metabolite?
A
D-arabotinol
25
(SG) What is the metabolic byproduct of the CLOSTRIDIA SPECIES?
3-4, dihydroxyphenolproprionate
| Cresol ??
26
(SG) Know the DIETARY POLYPHENOL breakdown products by bacteria
1. HIPPURATE
2. BENZOATE
3. Phenylacetate
4. Phenylproprionate (PPA)
27
(SG) What happens if there is a deficiency of zinc in reference to CYS? (Hint: what in urine?)
Can lead to DECREASED ABSORPTION of cys into protein, allowing SULFATE to spill into urine
28
(SG) Know what to supplement if there is xylene exposure (2) AND know what organic acid is elevated is there is xylene exposure
Supplement = B5 and Glycine
OA = 2MH (2-methylhippurate)
29
(SG) Know what 5 things you would use if there were elevations in urine PYROGLUTAMATE
1. NAC
2. GSH
3. Gly
4. Tau
5. Mg
30
(SG) what does the presence of ALPHA-HYDROXYBUTYRATE in the urine cause (hint: increased creation of?)
A shift from transmethylation to transulfuration and an increased creation of GLUTATHIONE
31
(SG) What is the main thing that elevations in urinary GLUCARATE indicate?
Impaired HEPATIC DETOXIFICATION
| And toxic exposure to pesticides, herbicides, drugs etc
32
(SG) Elevations in ammonia due to urea cycle dysfunction leads to a creation of which organic acid?
OROTATE
33
(SG) What can you use to treat the OA elevation to improve urea cycle function? (X4)
1. A- KG
2. Arginine
3. Magnesium
4. Aspartic acid
34
(SG) Homogentisate may be elevated from excess supplementation of which amino acid?
TYROSINE
35
(SG) What is the DNA oxidative damage marker?
8OH-dG (8-hydroxydeoxyguanosil)
36
(SG) Know the difference between HPLA and MeHPLA
HPLA = metabolite of tyrosine
MeHPLA = Importance cell growth inhibitor that CONTAINS HPLA which can be hydrolyze and freed
37
(SG) For 5HIAA = know the substrate, enzyme, and cofactors (3)
Substrate = SEROTONIN
Enzyme = MAO
Cofactors = Cu, Fe, B3/B2
38
(SG) What is the enzyme required for the creation of picolinate? (2 cofactors?)
Enzyme = 2-amino-3-carboxymuconate semialdehyde (ACMSD)
alpha-amino-beta-carboxymuconate-E-semialdehyde decarboxylase
(ACMSD) → Picolinate
Cofactors = Co, Fe
39
(SG) what is picolinate used for in the body? (X3)
1. Upregulates NO synthase
2. Activated inflammatory chemokines
3. PICO and QUIN work together to regulate leukocyte recruitment and distribution to damaged tissues during inflammatory responses
40
(SG) What is the cofactor for FORMINOTRANSFERASE (hint: X as Y)
B9 as THF
41
(SG) Know what the cofactor is for methylmalonyl-CoA mutate (and what do elevated MMA levels indicate?)
Cofactor = B12
Elevated MMA = B12 deficiency
42
(SG) What is the marker for biotin insufficiency?
Beta-hydroxyisovalerate or 2-OH-isovalerate
43
(SG) What do elevations in XANTHURENATE indicate?
B6 !!! deficiency
44
(SG) Dysfunctional branched chain ketoacid dehydrogenase complex will lead to an elevation of which urinary ketones? (X3)
Alpha-ketoisovalerate
Alpha-ketoisocaproate
Alpha-keto-beta-methylvalerate
45
(SG) What nutrient (and cofactor) is essential for mitochondrial beta-oxidation? (Hint it’s a shuttle)
L-CARNITINE (B5 cofactor)
46
(SG) The enzyme AST converts Aspartate to what?
OXALOACETATE
47
```
** T/F: In biochemistry, an enzyme ends with “ase,” a sugar ends in “ose,” and the ionized form of an organic acid ends in X?
A. Ite
B. Ate
C. -yl
D. - one
```
B. Ate like Butryate
48
** Elevations in lactate can indicate disruption of carbohydrate metabolism and the body’s need to regenerate vitamin ________ to keep the glycolytic pathway open
B3/NAD
49
** High urinary oxalates may occur from:
1. ) GI bacteria that produce it
2. ) Dietary ingestion of spinach, peanuts, beets, and chocolate
3. ) Absence of Oxalobacter formigenes
4. ) All of these responses
4. All of these response
50
** The marker for biotin insufficiency is:
1. ) Valine
2. ) alpha-keto-beta-methylvalerate
3. ) Beta-hydroxyisovalerate
4. ) alpha-ketoisovalerate
3. Beta-hydroxyvalerate
51
**Forminotransferase utilizes __________ to convert FIGLU to glutamate
1. ) 5,10-methylene THF
2. ) 5-formimino-THF
3. ) THF
4. ) 5MTHF
3. THF
52
** Norepinephrine and epinephrine can be degraded into _________ via the enzymes COMT and
1. ) Quinolinate
2. ) Hydroxyindoleacetate
3. ) Homovanillate
4. ) Vanilmandelate
4. VANILMANDELATE
53
** Homovanillate is the degradation produce of _________ catabolism
1. ) Serotonin
2. ) Dopamine
3. ) Epinephrine
4. ) Norepinephrine
2. Dopamine
54
** Homogentisate (HGA) may be elevated in urine from excess supplementation of which amino acid?
A. Cysteine
B. Methionine
C. Tyrosine
D. Tryptophan
C. Tyrosine
55
** __________ a metabolism expert of tyrosine, is a cell proliferation marker, and may indicate normal tissue growth or tumor growth (hint: H)
HPLA
56
** High ammonia levels from urea cycle dysfunction can lead to the accumulation of _ (a) . Treatment with (b) , may help the urea cycle function better and decrease urinary (a) levels.
1.) (a) Orotate, (b) Arginine, Mg++
2.) (a) Pyroglutamate, (b) NAC
3.) (a) Glucarate, (b) Arginine, Mn++
4.) (a) Sulfate, (b) NAC
High ammonia levels from urea cycle dysfunction can lead to the accumulation of _ (a) . Treatment with (b) , may help the urea cycle function better and decrease urinary (a) levels.
1. ) (a) Orotate, (b) Arginine, Mg++
2. ) (a) Pyroglutamate, (b) NAC
3. ) (a) Glucarate, (b) Arginine, Mn++
4. ) (a) Sulfate, (b) NAC
1. (A) orotate (b) Arg, Mg
57
** Deficiency of the mineral, ___________, can lead to decreased incorporation of cystine into protein, allowing sulfate to spill into the urine.
1. Copper
2. Zinc
3. Selenium
4. Iron
2. Zinc
58
** Alpha-hydroxybutyrate appears elevated in the urine when there has been a shift from trans- ___(a)___ to trans- ___(b)___ , to increase the creation of ___(c)___ .
1. ) (a) sulfuration, (b) methylation, (c) methionine
2. ) (a) sulfuration, (b) methylation, (c) GSH
3. ) (a) methylation (b) sulfuration, (c) methionine
4. ) (a) methylation, (b) sulfuration, (c) GSH
4.
59
** When the DNA oxidative damage marker, __________________, is seen elevated in urine with high QUIN and high HPLA, this indicates sustained inflammatory responses and increased cell proliferation rates causing increased reactive oxygen species (ROS), which lead to DNA damage via oxidation.
1. ) 8-hydroxy-2'-deoxyguanosine (8-OHDG)
2. ) 2-Methylhippurate
3. ) Homogentisate
4. ) Glucarate
1.
60
** High urinary _____________, a marker for phase I and II liver detoxification, may indicate exposure to pesticides, petrochemicals, alcohol and drugs.
1. ) Pyroglutamate
2. ) 2-Methylhippurate
3. ) Alpha-Hydroxybutyrate
4. ) Glucarate
4.
61
** Elevations in benzoate and not hippurate indicate:
1. ) Difficulty with phase II conjugation
2. ) There is a need for Glycine
3. ) All of these responses
4. ) There is a need for B5
3.
62
** Elevations in __(a) _ from xylene exposure may warrant supplementation with _(b) to assist cytochrome P450 oxidase enzymes and phase II conjugation reactions.
1. ) (a) 2-Methylhippurate, (b) NAC and B3
2. ) (a) Homogentisate, (b) NAC and P5P
3. ) (a) 2-Methylhippurate, (b) Glycine and B5
4. ) (a) Homogentisate, (b) Glycine and B3
3.
63
** Elevation in urinary pyroglutamate indicates that supplementation with ________ may be beneficial.
```
NAC
Glycine
GSH
Antioxidants
Mg
```
64
** High levels of suberate, adipate and ethylmalonate can be reduced with vitamin ________, which is utilized by the flavoprotein enzyme Fatty Aycl-CoA Dehydrogenase. (Hint: B...)
B2
65
** Isocitrate dehydrogenase converts isocitrate -> a-KG with the assistance of: (3 cofactors?)
B3/NAD
Mg
Mn
66
** The central energy pathways begin with digestion and assimilation of fats, carbohydrates, and proteins. Long chain FA require the use of the _________ shuttle to help them enter the mitochondrial for beta-oxidation.
CARNITINE
67
** A reaction that forms intermediates in a metabolic pathways like in the TCA cycle from precursors that are not part of the original metabolic pathway is considered a(n) ___________ reaction.
Anaplerotic reaction
68
** High urinary D-Lactate may indicate a person:
1. ) Is ingesting too much Lactobacillus acidophilus or L. plantarum
2. ) Has short bowel syndrome
3. ) Is ingesting too many carbohydrates
4. ) All of these responses
4
69
** Poor absorption of the amino acid, ___(a)___, can lead to accumulation of the dysbiosis metabolite ___(b)____.
1. ) (a) Tyrosine, (b) Tricarballylate
2. ) (a) Tryptophan, (b) Indican
3. ) (a) Tryptophan, (b) Tricarballylate
4. ) (a) Tyrosine, (b) Indican
2
70
** Bacterial ingestion of dietary polyphenols can lead to the creation of the intestinal dysbiosis markers _______________.
1. ) D-Arabinitol, Dihydroxyphenylpropionate
2. ) Cresol, Hydroxybenzoate, D-Arabinitol
3. ) Tricarballylate, D-Lactate
4. ) Benzoate, Hippurate, Phenylacetate, Phenylpropionate
4
71
Clostridia species, like C. difficile, can lead to elevations in several microbial dysbiosis markers such as:
1. ) Phenylpropionate
2. ) 3,4-Dihydroxyphenylpropionate
3. ) All of these responses
4. ) p-Cresol
3
72
** Elevations in lactate can indicate disruption of carbohydrate metabolism and the body's need to regenerate the vitamin _____________ to keep the glycolytic pathway functional
1. ) NADH
2. ) NADPH
3. ) FADH
4. ) NAD
4 (NAD = B3)
73
** Copper deficiency can lead to reduced activity of dopamine beta-monooxygenase (DBM), which converts dopamine -> norepinephrine. Hence, in copper deficiency you can find elevated urinary ____(a)____ and reduced urinary ____(b)_____ .
1. ) (a) VMA, (b) HVA
2. ) (a) HVA, (b) 5HIAA
3. ) (a) 5HIAA, (b) VMA
4. ) (a) HVA, (b) VMA
4
74
** High protein diets increase activity of ACMSD (alpha-amino-beta-carboxymuconate-E-semialdehyde decarboxylase), which converts 2-amino-3-carboxymuconate into the organic acid __________________ and leads to the upregulation of NO synthase, shifts Tryptophan metabolism away from B3 formation, and leads to activation of inflammatory chemokines.
1. ) Quinolinate
2. ) Kynurenate
3. ) Nicotinate
4. ) Picolinate
4
75
** The ratio of kynurenate (KYNA) to quinolinate (QUIN) is important because:
1. ) KYNA blocks NMDA receptors reducing glutamate excitotoxicity
2. ) KYNA activates NMDA receptors leading to increased glutamate toxicity
3. ) QUIN block NMDA receptors reducing glutamate excitotoxicity
4. ) QUIN activates NMDA receptors leading to reduced glutamate toxicity
1.
76
(SG) How can cu deficiency affect urinary HVA and VMA values?
Need Cu to convert DOPAMINE to NOREPINEPHRINE
Without Norepinephrine, can’t make VMA
Low Cu = HIGH HVA, Low VMA
77
(SG) Know all the cofactors for the electron transport chain (5)
1. Fe
2. Carnitine
3. CoQ10 (ubiquitol)
4. B2
5. B3
78
** High urinary D-Lactate may indicate a person:
1. ) Is ingesting too much Lactobacillus acidophilus or L. plantarum
2. ) Has short bowel syndrome
3. ) Is ingesting too many carbohydrates
4. ) All of these responses High urinary D-Lactate may indicate a person:
1. ) Is ingesting too much Lactobacillus acidophilus or L. plantarum
2. ) Has short bowel syndrome
3. ) Is ingesting too many carbohydrates
4. ) All of these responses
4
79
** Sugars ingested by yeast and fungi produce the intestinal dysbiosis marker:
1. ) 3,4-DHPP
2. ) D-Arabinitol
3. ) Tricarballylate
4. ) D-Lactate
2.
80
** Catechol-O-methyltransferase (COMT) utilizes _________ and _________ to degrade the catecholamines dopamine, norepinephrine, and epinephrine.
1. ) Cu++ and Fe++
2. ) NAD and Mg++
3. ) NAD and P5P
4. ) SAMe and Mg++
4
81
** It is important to note that elevations in Xanthurenate indicate that you should NOT supplement with any amino acids till you have first supplemented with vitamin _______ because transamination function is impaired.
1. ) B3
2. ) B6
3. ) B5
4. ) B2
2
82
** Catabolism of several AA and odd-chain FA eventually yields methylmalonyl-CoA (MMA). MMA conversion via the enzyme methylmalonyl-CoA mutase requires the vitamin:
1. ) B12
2. ) B2
3. ) B3
4. ) B6
1
83
** Elevation of the kynurenin pathway constituent 3-hydroxykynurenin leads to its side conversion into xanthurenate, which spills into the urine and can indicate insufficiency of vitamin ___________.
1. ) B6
2. ) B5
3. ) B3
4. ) B2
1
84
** Beta-hydroxybutyrate is a ketone body formed in diabetes (from the failure to utilize glucose appropriately) or from a low carbohydrate diet increasing dependency on FA oxidation. Both scenarios lead to a spill over of acetyl-CoA, which gets converted into beta-hydroxybutyrate. Treatment with what two minerals can help support the action of insulin and rebalance carbohydrate metabolism?
1. ) Magnesium and Copper
2. ) Zinc and selenium
3. ) Chromium and vanadium
4. ) Manganese and iron
3
85
** Citrate, Cis-Aconitate, and Isocitrate are located at the beginning of the TCA cycle. Elevations in cis-aconitate can indicate dysfunction of the enzyme aconitase, which requires the mineral ______________ and cysteine.
1. ) Se++
2. ) Zn++
3. ) Cu++
4. ) Fe++
4
86
** Insufficiency of vitamins B1, B2, B3, and B5 can lead to reduced enzymatic function of branched chain ketoacid dehydrogenase complex and causing increased urinary:
1. ) All of these responses
2. ) alpha-ketoisovalerate
3. ) alpha-keto-beta-methylvalerate
4. ) alpha-ketoisocaproate
1
87
** High levels of suberate, adipate and ethylmalonate can be reduced with vitamin ________, which is utilized by the flavoprotein enzyme Fatty Aycl-CoA Dehydrogenase.
B2
88
(SG) What are the 2 enzymes that initiate the kynurenine pathway? (Heme v not?)
Trp-2,3-doixygenase TDO (HEME)
Indoleamine-2,3-deoxygenase IDO
CLINICAL BIOCHEMISTRY
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