Quiz 1 LOs Flashcards

1
Q

What is the rate limiting enzyme of glycolysis?
What 2 molecules activate it?
What 2 molecules inhibit it?

A

PFK-1
ACTIVATE: AMP, F-2,6-BP

INHIBIT: ATP, citrate

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2
Q

Which of the following glucose transporters is located in the liver with the lowest affinity, and which is regulated by insulin?

GLUT1
GLUT2
GLUT3
GLUT4

A

GLUT2 is in liver with lowest affinity

GLUT4 is regulated by insulin

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3
Q

Pyruvate kinase is a major regulated step in glycolysis. What 2 molecules stimulate activation, and what 3 molecules inhibit its activity?

Note: Pyruvate kinase works and you’re “in”, but when it’s off “GAA”

A

ACTIVATE: Insulin, F-1,6-BP

INHIBIT: Alanine, ATP, Glucagon

Note: Pyruvate kinase works and you’re “in”, but when it’s off “GAA”

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4
Q

Which of the two hormones is associated with dephosphorylation?

A. Glucagon
B. Insulin

A

Insulin

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5
Q

Taurus’ are Pretty Fucking Kind, but Tarui disease is not. PTs present with exercise induced cramps, hemolytic anemia, high bilirubin and jaundice. Teuri disease is caused by a deficiency in what major enzyme of glycolysis?

A

PFK-1

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6
Q

T/F: It seems like insulin and phosphatases is always vs. glucagon and cAMP/PKA

A

True

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7
Q

Pyruvate can be converted to alanine through what two metabolic processes?

A

Gluconeogenesis

Urea cycle

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8
Q

About 95% of glycolysis related anemias are associated with defects in what enzyme?

A

Pyruvate kinase

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9
Q

What are two major clinical markers for hemolytic anemia?

A. Lactate dehydrogenase, conjugated bilirubin
B. Pyruvate kinase, PFK-1
C. Lactate dehydrogenase, unconjugated bilirubin
D. None of the above

A

Lactate dehydrogenase, unconjugated bilirubin

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10
Q

Fanconi-Bickel syndrome is a “TWO-NAME SYNDROME”, with a lot of words to TRANSPORT. Presentation includes hepatosplenomegaly, failure to thrive, and resistant rickets. What deficiency is causes this two-name syndrome?

A

GLUT2 defect that prevents transport of glucose, galactose, and fructose to be transported

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11
Q

Which of the following, is an activator for all of the major regulatory steps of gluconeogenesis that include the following enzymes: Pyruvate carboxylase, PEP carboxykinase, F-1,6-Biphosphatase, and Glucose-6-phosphatase?

A. AMP
B. Cortisol
C. Citrate
D. ADP

A

Cortisol

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12
Q

What is the rate limiting enzyme of gluconeogenesis?
What are the 2 aCCtivators?
What are its 2 inhibitors?

A

Fructose-1,6-biphosphatase
ACTIVATORS: Citrate, Cortisol

INHIBITORS: AMP, F2,6BP

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13
Q

What are the only 3 activators of PEP carboxykinase that stimulate its activity in gluconeogenesis?

A

Cortisol
Glucagon
Thyroxine

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14
Q

What are the 2 activators of pyruvate carboxylase and its only ADept inhibitor as it acts in gluconeogenesis?

A

ACTIVATOR: Cortisol, Acetyl CoA

INHIBITOR: ADP

NOTE: pyruvate carboxylase has an ADept inhibitor

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15
Q

If F2,6BP activates PFK-1 in glycolysis then it makes sense that F2,6BP inhibits what enzyme in gluconeogenesis?

A

Fructose-1,6-biphosphatase

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16
Q

Which of the following enzymes is the only one to be found in the mitochondria and utilizes biotin (B7)?

A. PEP carboxykinase
B. Fructose-1,6-biphosphatase
C. Glucose-6-phosphatase
D. Pyruvate carboxylase

A

Pyruvate carboxylase

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17
Q

Tarui disease is caused by a deficiency in PFK-1. What other disease is similar to Tarui disease in gluconeogenesis?

A

Fructose-1,6-biphosphatase deficiency and Tarui disease present similaraly

Lactic acidosis, hypoglycemia

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18
Q

Explain how glucose is made into fructose in cells that have sorbitol dehydrogenase in the Polyl pathway. Include intermediates and enzymes used.

A

Glucose + Aldose reductase –> sorbitol + sorbitol dehydrogenase —> fructose

Cells lacking sorbitol dehydrogenase: Kidney, retina, schwann cells

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19
Q

Fructose w/ _________ makes F6P, and enters in glycolysis. While fructose with _______ makes F1P, skipping major regulatory step and entering glycolysis at intermediate Glyceraldehyde 3 phosphate

A

Hexokinase

Fructokinase

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20
Q

In fructose metabolism, what two enzymes bypass the major regulatory step of glycolysis?

A

Fructokinase

Triose Kinase

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21
Q

Galactosemia has non-classical and classical versions depending on the deficinent enzyme. What is the deficient enzyme in either version that leads to the buildup of galactose or galactitol causing cataracts?

A

Classical: GALT/glacatose 1P uridyltransferase

Non-classical: Glactokinase

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22
Q

T/F: Galactose is one step away from Galactitol via aldolase reductase

A

True

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23
Q

What is the rate limiting enzyme of the PPP?

A

Glucose-6-phosphate dehydrogenase

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24
Q

Which of the following is the likely result with GSD 0? Bonus: what enzyme is deficient?

A. Long chains with few branches
B. Short chains with many branches
C. Free glucose unable to be converted to glycogen
D. None of the above

A

Free glucose unable to be converted to glycogen

Enzyme: Glycogen synthase (rate limiting enzyme)

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25
Q

Which of the following is the likely result with Andersen disease (GSD IV)? What enzyme is deficient

A. Long chains with few branches
B. High number of short branches
C. Free glucose unable to be converted to glycogen
D. None of the above

A

Long chains with few branches

Enzyme: Glucosyl (4:6) transferase

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26
Q

What is the rate limiting enzyme of glycogenolysis and glycogenesis?

A

Glycogenesis: Glycogen synthase

Glycogenolysis: Glycogen phosphorylase

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27
Q

Which of the following is the likely result with Cori disease (GSD III)? What is the deficient enzyme?

A. Long chains with few branches
B. High number of short branches
C. Free glucose unable to be converted to glycogen
D. None of the above

A

High number of short branches

a-1,6-glucosidase

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28
Q

In terms of phosphorylation, which form of glycogen synthase is active and which form of glycogen phosphorylase is active?

A

DEphosphorylated glycogen synthase is active

Phosphorylated glycogen phosphorylase is active

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29
Q

T/F: Glucagon does not act on the muscle

A

True

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30
Q

ATP citrate lyase is the enzyme responsible for converting citrate back to acetyl CoA and OAA once out in the cytosol in FA synthesis. What are citrate lyase’s 2 activators, and what are the 2 inhibitors?

A

ACTIVATORS: Glucose, Insulin

INHIBITORS: PUFA, leptin

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31
Q

T/F: Oxaloacetate is unable to enter and exit mitochondria directly

A

True

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32
Q

In fatty acid synthesis, which of the following acts as an inhibitor in every major phase?

A. PUFA
B. Leptin
C. Insulin
D. Glucagon

A

PUFA

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33
Q

T/F: Carboxylase enzymes generally utilize biotin as a cofactor

A

True

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34
Q

What is the rate limiting step and enzyme in fatty acid synthesis?

A

Acetyl CoA –> Malonyl CoA

Acetyl-CoA carboxylase

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35
Q

The presence of malonyl coA inhibits the function of what rate limiting enzyme in fatty acid degredation, in order to prevent them from occuring at the same time?

A

Carnitine acyl transferase

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36
Q

Phase III of fatty acid synthesis is chain formation where 2carbon units are added to the fatty acyl chain in 7 reactions to form palmitate. What is the name of the enzyme complex that carries this out?

A

Fatty Acid Synthase Complex

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37
Q

Fatty acid synthase is a complex of 2 identical dimers made up of 7 enzymes and what additional protein with a flexible arm?

A

Acyl Carrier protein

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38
Q

What 2 hormones/hormone types stimulate the activity of Fatty Acid Synthase Complex?

A

Insulin

Glucocorticoid hormones

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39
Q

Because the brain requires longer chain fatty acids (C18-24), it is necessary for palmitate to be lengthened. This addition can only happen in 2 different locations, and each use their own carbon donor plus NADPH for reducing power. What are the 2 locations where this occurs, and what are their respective carbon donors?

A

Smooth ER uses Malonyl CoA

Mitochondria uses Acetyl CoA

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40
Q

What are the 4 human desaturases and where do they each present a new double bond?

A

delta4: 4-5
delta5: 5-6
delta6: 6-7
delta9: 9-10

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41
Q

TAGs are made in through de novo synthesis in all of the following locations except for which, which is where dietary TAGs are processed?

A. Hepatocytes
B. Adipocytes
C. Intestinal cells
D. None of the above

A

Intestinal cells

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42
Q

What is the name of the lipoprotein that contaiins apolipoproteins and other lipids synthesized from the small intestines? These lipoproteins later enter the lymphatic system through the thoracic duct.

A. Chylomacroons
B. VLDLs
C. LDLs
D. Chylomicrons

A

Chylomicrons

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43
Q

In the de novo synthesis of TAGs made in the liver glucose and glycerol combine to form glycerol-3-phosphate. This glycerol-3-phosphate is used as the backbone and combined with FFAs to form TAGs. These TAGs are packaged into lipoproteins that are called:

A. Chylomacroons
B. VLDLs
C. LDLs
D. Chylomicrons

A

VLDLs

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44
Q

T/F: TAG synthesis in adipocytes is promoted by excess carbohydrates and fats

A

True

45
Q

De novo synthesis of TAGs in adipose tissue utilizes what enzyme that combines chylomicrons and VLDLs?

A

Capillary Lipoprotein Lipase

46
Q

The breakdown of TAGs involves the work of 3 major lipases. What are their names in the order in which they are used?

A
  1. Hormone Sensitive Lipase
  2. Lipoprotein lipase
  3. Monoacylglycerol lipase
47
Q

Unlike short and medium chain FAs, long and very long chain FAs cannot diffuse into the mitochondria and must be actively transported. What is the name of the transporter responsible for shuttling the LCFA and VLCFA into the mitochondria?

A

Carnitine shuttle

48
Q

What is the rate limiting enzyme of the carnitine shuttle during fatty acid oxidation? What is it inhibited by

A

CArnitine palmitotransferase I

Inhibited by Malonyl CoA

49
Q

Ketone bodies are water-soluble, acidic compounds that are only produced in the liver. What are the names of 3 ketone bodies?

A

Acetoacetate
B-hydroxybutyrate
Acetone

50
Q

What are the 3 purposes of nutrition?

A
  1. Promote growth and development
  2. Provide energy
  3. regulate metabolism
51
Q

Which of the following proteins is stabilized through hydrophobic interactions with the membrane?

A. Integral membrane proteins
B. Peripheral proteins
C. Lipid anchored proteins
D. None of the above

A

Integral membrane proteins

52
Q

Which of the following proteins is loosely bound to membrane through electrostatic interactions?

A. Integral membrane proteins
B. Peripheral proteins
C. Lipid anchored proteins
D. None of the above

A

Peripheral proteins

53
Q

Lipid anchored proteins are tethered to the plama membrane through which of the following interactions?

A. Electrostatic
B. Hydrophobic
C. Covalent
D. Ionic

A

Covalent

54
Q

All of the following phospholipids are normally found in the outerleaflet except for which of the following which is only found during apopotosis?

A. Phosphatidylcholine
B. Glycolipids
C. Sphingomyelin
D. Phosphotidylserine

A

Phosphotidylserine

55
Q

Which of the following diseases leads to an accumulation of sphingomyelin in the lysosomes of the liver, spleen, CNS, or bone marrow? What is the deficient enzyme?

A. Fanconi-Bickel syndrome
B. Niemann-Pick disease
C. Andersen Disease
D. Cori disease

A

Niemann-Pick disease

deficient in Acid Sphingomyelinase which breaks down sphingomyelin into ceramide and phosphorylcholine

56
Q

During blood transufsions why is it important to cross match? In other words what are you ensuring doesn’t interact between the donor and recipient?

A

Cross-matching is necessary to ensure the donor’s blood antigens doesn’t interact with the recipients antibodies in the plasma

57
Q

In terms of ABO blood system, antibodies are found in the ______ while antigens are found in the _____.

A

plasma

RBC surface

58
Q

In order to be a universal donor, what is required of your blood. What is required for a universal acceptor?

A

Universal donors have no blood group antigens
- like Type O w/ H antigen

Universal acceptors have no antibodies in their plasma
- like Type AB

59
Q

A patient is diagnosed with advanced liver cirrohsis, and another patient with Beta-lipoproteinanemia. Oddly enough, you notice high levels of excess free cholesterol in the blood. Which of the following issues might you look out for when analyzing the blood further?

A. Spur cell anemia
B. Neimann-pick disease
C. Acanthocytes
D. All of the above
E. A and B
F. A and C
A

F.
Spur cell anemia
Acanthocytes

60
Q

A patient presents with renal cholic (abdominal pain associated with kidney stones). PT comes back with a positive nitroprusside test and you diagnose them with Cystinuria. If you were to collect samples of crystals passed by this patient, what might you find they are made of?

A

Dimeric AA: Cystine

Dibasic AAs: Ornithine, Lysine, Arginine

CORK

61
Q

PT presents with cerebellar ataxia, nyastygmus, photodermatitis, and photosensitivity. You record low levels of melatonin, serotonin, and niacin. What is the most likely disease, and what AA is likely not being transported?

A

Hartnup disease

Tryptophan: an important precursor for serotonin, melatonin, niacin

62
Q

PT presents with CHF, and you wish to better control this. You select a drug that inhibits the Na/K ATPase that would lead to an increase in intracellular [Na] and impair the secondary NCX which increase [Ca] causing a stronger contraction in cardiac muscle. What is the name of the drug you most likely selected?

A

Digoxin

Ouabain

63
Q

T/F: Phosphatase removes a phosphate while phosphorylase is a type of phosphotransferase that adds an additional phosphate group originally from an inorganic compound

A

True

64
Q

In order to establish a proton gradient protons must be pumped from the ______ to the ________. This is done through the movement of electrons through the complexes

A

Matrix (low)

Inner membrane space (high)

65
Q

When the transfer of electrons is inhibited in the OxPhos 3 things happen. Which of the following is not one of them?

A. Decrease in proton pumping
B. Decrease in proton gradient
C. Increase in proton pumping
D. Inhibition of ATP synthesis

A

Increase in proton pumping

66
Q

Which of the following occurs when ATP synthase is uncoupled from ETC as seen in hybernating animals?

A. Proton leakage back into matrix
B. Inhibition of ATP synthase
C. Acceleration of TCA and e- transfer to O2
D. Heat production
E. All of the above
F. All but C
A

All of the above

67
Q

All of the following is true of the malate-aspartate shuttle except which of the following that is true of the glycerophosphate shuttle? What change would you make to make them all correct?

A. Operates in heart, liver, kidneys
B. Generates FADH2 in the inner mito-membrane
C. Allows entry of oxidant at Complex I
D. All the above are correct

A

Generates FADH2 in the inner mito-membrane

Malate-aspartate shuttle generates NADH in the mitochondrial matrix

68
Q

All of the following is true of the glycerophosphate shuttle except which of the following that is true of the malate-aspartate shuttle? What change would you make to make them all correct?

A. Operates in brain and muscle
B. Generates FADH2 in the inner mito-membrane
C. Allows entry of oxidant at Complex I
D. All the above are correct

A

Allows entry of oxidant at Complex I

Glycerophosphate shuttle allows entry of FADH2 at CoQ

69
Q

An excess in citrate favors which of the following processes?

A. Aerobic glycolysis (pyruvate –> acetyl CoA)
B. TCA
C. Non-oxidative glycolysis (pyruvate–> lactate)
D. Activity of PDC

A

Non-oxidative glycolysis (pyruvate–> lactate)

70
Q

Which of the following peptidases would clip at the end of a peptide at the C terminus?

A. Aminopeptidase
B. Carboxypeptidase
C. Endopeptidase
D. Enteropeptidases

A

Carboxypeptidase

71
Q

Which of the following peptidases is responsible for cleaving and activating zymogens in extracellular environment?

A. Aminopeptidase
B. Carboxypeptidase
C. Endopeptidase
D. Enteropeptidases

A

Enteropeptidases

72
Q

What AAs are ketogenic only, and which are both ketogenic and glucogenic?

A

Ketogenic only: KL

BOTH: WIFTY

73
Q

Patients with MSUD are likely to have what AA found in thier urine?

A

Branched Chain AA

Treated with thiamine (B1)

74
Q

PT presents with urine of a musty smelling odor. Analysis shows elevated levels of phenylactate and phenylacetate which are known to block neurotransmission and myelin formation. PT is diagnosed with PKU (Phenylketonuria). What AA is converted erroneously to cause this issue. What AA can be used to treat this issue to supplement the poorly processed AA?

A

Phenylalanine

Tyrosine

PKU is caused by defective activity og phenylalanine hydroxylase

75
Q

PLP is needed in transaminase or aminotransferases and is the active form of which of the following B vitamins?

A. B1
B. B2
C. B6
D. B12

A

B6

76
Q

T/F: Phenylalanine is converted to Tyrosine through the activity of phenylalanine hydroxylase

A

True

77
Q

In order for Tryptophan to make niacin, which of the following vitamins is needed?

A. B1
B. B3
C. B6
D. B12

A

B6

NOTE: B3 is niacin

78
Q

Melanin is a product of which of the following AAs?

A. Threonine
B. Tyrosine
C. Tryptophan
D. None of the above

A

Tyrosine

Dopamine, norepinephrin
Thyroid hormones
Melanin

79
Q

Serotonin and melatonin are products of which of the following AAs?

A. Threonine
B. Tyrosine
C. Tryptophan
D. None of the above

A

Tryptophan

80
Q

THe goal of the urea cycle is to remove nitrogen in the form of ammonia. In the brain ammonia is removed as what two AAs?

A

Glutamate

Glutamine

81
Q

B1

A

thiamine

82
Q

B2

A

riboflavin

83
Q

B3

A

niacin

84
Q

Pantothenic acid

A

B5

85
Q

Pyridoxine

A

B6

86
Q

B7

A

Biotin

87
Q

Folic acid

A

B9

88
Q

B12

A

Cobalamin

89
Q

Vitamin C

A

Ascorbate

90
Q

What are the 4 fat soluble vitamins

A

ADEK

91
Q

BeriBeri has two forms dry and wet. Deficiency in what vitamin leads to BeriBeri? Describe each form briefly.

A

Thiamine deficiency (B1)

Dry: Muscle wasting and partial paralysis
Wet: Peripheral edema and cardiac failure

92
Q

Accumulation of sorbitol causes cataracts

A

True

93
Q

A deficiency in what enzyme impairs the breakdown of medium chain FAs, often leading to secondary carnitine deficiency. The number of C8 fatty acids accumulate in the liver impairing urea cycle and increasing ammonia levels.

A

Medium chain acyl CoA dehydrogenase

94
Q

The degredation of very long chain FAs occurs in what cellular location and utilizes what enzyme in the first step?
This enzyme is specific to the organelle

A

Peroxisome

Acyl-CoA oxidase

95
Q

Uracil replaces what pyrimidine base in RNA?

A

Uracil replaces Thymine

96
Q

All of the following are true of xanthine oxidase, except for which of the following that is true of adenosine deaminase?

A. Converts hypoxanthine to xanthine
B. Target for gout treatment
C. Converts xanthine to uric acid
D. Excess leads to hemolytic anemia, and deficiency leads to SCID

A

Excess leads to hemolytic anemia, and deficiency leads to SCID

NOTE: Adenosine deaminase-performs an irreversible hydrolytic deamination making adenosine to inosine

97
Q

HGPRT is able to generate which of the following purines in the purine salvage pathway, while APRT is able to generate what?

A. AMP
B. IMP
C. GMP

A

HGPRT: IMP and GMP

APRT: AMP

98
Q

T/F: On pyrimidine synthesis the rate limiting step is the formation of carbomoyl aspartate

A

True

99
Q

Transaminases transfer an amino group to an a-ketoacid and are coupled with what co-enzyme? What B vitamin is the coenzyme derivative of?

A

PLP-pyridoxyl-5-phosphate

B6

100
Q

Which of the following enzymes is used to remove the NH4 from glutamine creating glutamate?

A. Glutaminase
B. Glutamine synthase

A

Glutaminase

101
Q

Which of the following enzymes is used to add an ammonia to glutamate to make glutamine?

A. Glutaminase
B. Glutamine synthase

A

Glutamine synthase

102
Q

What is the name of the enzyme responsible for removing the ammonia from Glutamine in the brain?

A

Glutamine synthase

103
Q

Removal of excess NH4 from the muscle requires what enzyme?

A. Glutaminase
B. Glutamine synthase
C. Alanine Transaminase
D. Aspartate transaminase

A

Alanine Transaminase

104
Q

What are the names of the 3 uncouplers?

A

DNP, aspirin, thermogenin (UCP-I)

105
Q

Arsenic attacks what complex in cellular metabolism?

A

E2 of pyruvate dehydrogenase complex

106
Q

T/F: Excess citrate reduces the activity of PDC causing favoribility of nonoxidative glycolysis, pushing increase pyruvate conversion to lactate

A

True

107
Q

Increase of citrate activates which of the following enzymes?

A. Pyruvate Dehydrogenase
B. PFK1
C. Acetyl CoA carboxylase
D. All the above

A

Acetyl CoA Carboxylase

  • enzyme produces acetyl CoA and Maloney’s CoA pushing lipid synthesis
108
Q

Transaminases like alanine transaminase or aspartate transaminase require what coenzyme?

A. Vitamin B6 derived PLP
B. Vitamin B12 cobalamine
C. Vitamin B7 biotin
D. Vitamin B5 pantothenic acid

A

Vitamin B6 derived PLP

PLP = pyridoxal phosphate