MCP Flashcards

1
Q

Sitosterolemia is caused by mutations in the _____ and _____ genes

A

ABCG5 and ABCG8

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2
Q

The ABC transporters deficient in Sitosterolemia are ABC transporters _____ and _____

A

Sterolin-1 and Sterolin-1

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3
Q

An inability to pump plant sterols can lead to _______

A

Sitosterolemia

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4
Q

Cholesterol synthesis occurs on the surface of the (cellular component) ______

A

Smooth ER

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5
Q

The two compounds added to Cholic acid and chenodeoxycholic acid to make them “conjugated” are ____ and ____

A

glycine and taurine

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6
Q

Primary bile acids are converted to secondary bile acids by the action of _____

A

intestinal bacteria

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7
Q

HMG CoA Reducatase is active when it is (phosphorylates/dephosphorylated)

A

dephosphorylated

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8
Q

The ____ protein binds cholesterol so that the SREBP protein can activate transcription

A

SCAP

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9
Q

Hormones insulin and thyroxine (up-regulate/down-regulate) expression of HGM CoA reductase

A

up-regulate

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10
Q

SLOS is a deficiency in ____ leading to impaired _____ synthesis

A

7DHC

impaired cholesterol synthesis

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11
Q

Mutations inABCG5and ABCG8geneswhichencodeABC transporters can lead to _______

A

Sitosterolemia

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12
Q

They key regulatory step of Cholesterol synthesis is catalyzed by the enzyme ________

A

HMG CoA Reductase

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13
Q

A deficiency in the enzyme cholesterol acyl transferase (ACAT) will result in (Over production of cellular cholesterol/Reduction of cellular cholesterol esters)

A

Reduction of cellular cholesterol esters

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14
Q

Apo ___ is unique to chylomicrons

A

Apo B48

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15
Q

Chylomicrons are assembled in (cells)_____

A

intestinal mucosal cells

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16
Q

Nacent chylomicrons are released into the (blood/lymph)

A

lymph

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17
Q

LPL binds Apo ____

A

C II

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18
Q

VLDL is produced by the _____

A

liver

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19
Q

VLDL is converted to LDL in the _____

A

blood

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20
Q

Apo E(1/2/3/4) binds poorly

A

Apo E2

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21
Q

Apo E(1/2/3/4) associated with hyperlipoprotienemia

A

Apo E2

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22
Q

Apo E(1/2/3/4) associated with alzheimers

A

Apo E4

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23
Q

If there is excess cholesterol, it can be stored as an ester by the action of (enzyme) ______

A

ACAT

Acyl CoA: cholesterol acyl transferase

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24
Q

Excess cholesterol gets stored as ______

A

cholesterol esters

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25
Q

Apo ___ is an LPL activator, while Apo ____ is an LPL inhibitor

A

C II

C III

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26
Q

HDLs are the only carriers of Apo ____ and Apo ____

A

A I and A II

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27
Q

Tri-Glycerides in VLDL are exchanged with Cholesterol Esters in HDL by the action of _____

A

CEPT

Cholesterol ester transfer proteins

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28
Q

Apo E and Apo C II are put onto other lipoproteins from circulating _____

A

HDL

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29
Q

Excess cholesterol is returned to the liver via ____

A

HDL

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30
Q

Tangier disease is a deficiency in _____ which leads to a lack of HDL particles

A

ABCA1

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31
Q

Scavenger Receptor B1 (SR-B1) helps to clear (VLDL/LDL/HDL/Chylomicrons)

A

HDL

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32
Q

The enzyme that converts cholesterol to cholesterol esters so that it may be stored in HDL is _____

A

LCAT

Lecithin—cholesterol acyltransferase

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33
Q

Cholesterol is moved into the mitochondria for steroid synthesis by the transporter _____

A

StAR

steroidogenic acute regulatory protein

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34
Q

The rate limiting step in the conversion of cholesterol to steroids is catalyzed by the enzyme _____

A

CYP11A

a cytochrome p450 mixed function oxidase

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35
Q

CYP11A is located at the ______

A

inner mitochondrial membrane

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36
Q

Corticotropin releasing hormone (CRH) induces __________ which induces glucocorticoid cortisol secretion

A

ACTH

Adrenocorticotropic hormone

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37
Q

High cortisolconcentrations (promotes/inhibits) Corticotropin releasing hormone (CRH)

A

inhibits (negative feedback)

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38
Q

Function of Aldosterone:

A

Increase Na+ (and H2O) uptake at kidney tubules

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39
Q

Angiotensinogen is converted to angiotensin I by ______

A

Renin

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40
Q

Angiotensin I is converted to angiotensin II by ______

A

ACE (angiotensin converting enzyme)

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41
Q

Renin dependent hypertension is treated by ______

A

ACE inhibitors

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42
Q

Steroid hormones are secreted by the (liver/gonads/adrenal cortex/thyroid)

A

adrenal cortex

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43
Q

Hormone that acts on intestines and causes increased calcium absorption is _____

A

calcitrol

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44
Q

The active form of vitamin D in the body is (calcidiol/calcitriol)

A

calcitriol

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45
Q

Calcidiol is converted to calcitriol in the ______

A

kidneys

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46
Q

Calcium levels are monitored by the (organ) ______

A

parathyroid

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47
Q

_____ stimulates intestinal absorption of calcium by binding the VitaminDreceptor(VDR)

A

calcitrol = 1,25-diOH-D3

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48
Q

calcitrol (1,25-diOH-D3) binds the ____ in the cytoplasm of intestinal cells

A

VitaminDReceptor(VDR)

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49
Q

On an enterocyte, _______ mediates transport of calcium across the apical membrane

A

Calbindin-D9K

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50
Q

Movement of calcium into the epithelial cell is mediated by ______

A

TRVP5

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51
Q

The MEOS consists of a cyt P450 enzyme called ______

A

CYP2E1

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52
Q

In the liver EtOH is converted to Acetaldehyde by _____

A

ADH

Acetaldehyde deyhydrogenase

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53
Q

In the liver Acetaldehyde is converted to Acetate by _____

A

ALDH

acetaldehyde dehydrogenase

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54
Q

The most abundant isozyme of ADH is (ADH1/ADH2/ADH3)

A

ADH1

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55
Q

The isozyme of ADH best for long chain alcohols is (ADH1/ADH2/ADH3)

A

ADH3

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56
Q

(ALDH1/ALDH2) is abundant in the mitochondria

A

ALDH2

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57
Q

(ALDH1/ALDH2) is abundant in the cytoplasm

A

ALDH1

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58
Q

Treating alcoholics with disulfiram inhibits the enzyme ______

A

ALDH

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59
Q

Oxidation of acetylaldehyde occurs in the (organelle) ______

A

mitochondria

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60
Q

The A vitamins have important functions in _____

A

vision

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61
Q

Excess vitamin A accumulates in the (organ) _____

A

liver (ito cells)

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62
Q

Keratomalacia can occur with a deficiency in which vitamin?

A

Vitamin A

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63
Q

Deficiency of Vitamin D can lead to _____ in children and ________ in adults

A

Rickets

osteomalacia

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64
Q

A deficiency in this vitamin results in easy bruising and bleeding

A

Vitamin K

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65
Q

Vitamin K has an essential role in modifying ________, part of the clotting cascade

A

Prothrombin

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66
Q

Which vitamin functions as an antioxidant?

A

Vitamin E

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67
Q

Which vitamin is present in all cell and organelle membranes?

A

Vitamin E

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68
Q

A severe deficiency in this vitamin can lead to impaired collagen formation and scurvy

A

Vitamin C

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69
Q

Synthesis of epinephrin in adrenal glands requires which vitamin?

A

Vitamin C

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70
Q

Which B vitamins are hematopoietic?

A

B9: Folate
B12: Cobalamine

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71
Q

Which B vitamins are energy releasing?

A

B1 Thiamine
B2 Riboflavin
B3 Niacin

B5 Pantothenic Acid
B6 Pyridoxine
B7 Biotin

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72
Q

B1 is ____

A

Thiamine

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73
Q

B2 is ____

A

Riboflavin

74
Q

B3 is _____

A

Niacin

75
Q

B5 is _____

A

Pantothenic acid

76
Q

B6 is _____

A

Pyridoxine

77
Q

B9 is _____

A

Folic Acid

78
Q

B12 is _____

A

Cobalamine

79
Q

A severe deficiency in ____ causes Beri Beri

A

Thiamine B1

80
Q

Which vitamin is a precursor for NAD and NADP?

A

Niacin B3

81
Q

Which vitamin is a precursor for FAD and FMN?

A

Riboflavin B2

82
Q

A deficiency in which vitamin will lead to pellagra?

A

Niacin

83
Q

Which vitamin is required for synthesis of CoA

A

Pantothenic acid B5

84
Q

This vitamin is a precursor to for PLP

A

Pyridoxine B6

85
Q

This vitamin is required for synthesis of GABA and Heme

A

Pyridoxine B6

86
Q

Which B vitamin is a precursor to tetrahydrafolate?

A

Folate B9

87
Q

Inadequate levels of vitamin _____ can lead to neural tube defects

A

B9, Folate

88
Q

Pernicious anemia refers specifically to a deficiency in _____

A

B12 due to a lack of intrinsic factor

89
Q

Menkes’ disease results from an inability to transport _____

A

Cu

90
Q

One of the earliest symptoms of a zinc deficiency is _____

A

dermatitis

91
Q

Which mineral is important for insulin to bind its receptor?

A

chromium

92
Q

Deficiency in this mineral an lead to an enlarged thyroid that becomes a goiter

A

Iodine

93
Q

Which mineral is a component of glutathione peroxidase?

A

selenium

94
Q

An overload in in iron can lead to a disease called _______

A

hemochromatosis

95
Q

Copper is a critical co-factor for (mineral) ______ absorbtion

A

iron

96
Q

Which vitamin is imperative for blood coagulation?

A

Vitamin K

97
Q

If intestinal bacteria are missing either due to antibiotics or in new borns, which vitamin may be deficient?

A

Vitamin K

98
Q

What are the three most common vitamin deficiencies in alcoholics?

A

Folate
B6
Thiamine

99
Q

The “wet brain” associated with B1 deficiency in alcoholics is formally called ______

A

Wernicke-Korsaoff Syndrome

100
Q

Folate is important for (cell component) _____ synthesis

A

DNA

101
Q

Most superoxide generation occurs within the ______

A

mitochondria

102
Q

The most dangerous free radical is ______

A

*OH free radical

103
Q

Produced by Fenton reaction:

O2-/OH-/NO*/H2O2/ONOO-

A

hydroxyl radical

104
Q

Produced from hydrogen peroxide by ionizing radiation

O2-/OH-/NO*/H2O2/ONOO-

A

hydroxyl radical

105
Q

Produced form the mitochondrial ETC

O2-/OH-/NO*/H2O2/ONOO-

A

Superoxide

106
Q

Produced in the oxidation of hemoglobin

O2-/OH-/NO*/H2O2/ONOO-

A

Superoxide

107
Q

Produced by nitric oxide synthase

O2-/OH-/NO*/H2O2/ONOO-

A

Nitric oxide

108
Q

Produced by the reaction between nitric oxide and superoxide

O2-/OH-/NO*/H2O2/ONOO-

A

ONOO-

109
Q

Produced by myeloperoxidase (MPO)

O2-/OH-/NO*/HClO/ONOO-

A

HClO

110
Q

Substrate for myeloperoxidase

NADP+/NAD+/NADPH/*O2-/H2O2

A

hydrogen peroxide

111
Q

Product for superoxide dismutase reaction

NADP+/NAD+/NADPH/*O2-/H2O2

A

hydrogen peroxide

112
Q

Substrate for Glutathione peroxidase

NADP+/NAD+/NADPH/*O2-/H2O2

A

hydrogen peroxide

113
Q

Product of nitric oxide synthase

NADP+/NAD+/NADPH/*O2-/H2O2

A

NADP+

114
Q

Reacts with nitric oxide to produce peroxinitrite

NADP+/NAD+/NADPH/*O2-/H2O2

A

Super oxide

115
Q

Substrate for respiratory burst oxidase

NADP+/NAD+/NADPH/*O2-/H2O2

A

NADPH

116
Q

Reacts with Fe2+ to produce hydroxyl radical

NADP+/NAD+/NADPH/*O2-/H2O2

A

H2O2

117
Q

Product of glucose-6-phosphate dehydrogenase reaction

NADP+/NAD+/NADPH/*O2-/H2O2

A

NADPH

118
Q

Some cases of ALS have been associated with this enzyme

G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione

A

S.O.D.

Superoxide dismutase

119
Q

Keeps sulfahydryls of proteins reduced

G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione

A

Reduced Glutathione

120
Q

Chronic granulomatous disease is associated with mutations in this membrane bound complex
(G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione)

A

Nox

NADPH oxidase

121
Q

With deficiency in this enzyme you will get hemolytic anemia after eating fava beans
(G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione)

A

Glucose-6-phosphate dehydrogenase

122
Q

*O2- is a product of this enzyme

G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione

A

Nox

NADPH oxidase

123
Q

Decomposes hydrogen peroxide

G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione

A

Catalase

124
Q

Reducing protein disulfides

Fe3+/Fe2+/reduced glutathione/oxidized glutathione

A

reduced glutathione

125
Q

Reacts with H2O2 in Fenton reaction

Fe3+/Fe2+/reduced glutathione/oxidized glutathione

A

Fe2+

126
Q

Produced by reactions with H2Os in Fenton reaction

Fe3+/Fe2+/reduced glutathione/oxidized glutathione

A

Fe3+

127
Q

Presenting as the dimeric GSSG

Fe3+/Fe2+/reduced glutathione/oxidized glutathione

A

oxidized glutathione

128
Q

Leptin (increases/decreases) appetite

A

decreases

129
Q

The protein UCP1 (uncoupling protein) is found in which type of fat?

A

brown fat (and beige fat)

130
Q

Which protein in brown fat is responsible for proton leaking across the inner mitochondrial membrane?

A

UCP1 (uncoupling protein 1)

131
Q

2,4 dinitrophenol (DNP) can function as a ______ to allow protons to leak across the membrane

A

uncoupler

132
Q

UCP1 (thermogenin) can be activated by which adrenergic receptor?

A

β3

133
Q

Under normal physiological conditions, KSR2 (kinase suppressor of Ras2) works to (increase/decrease) glucose and fatty acid oxidation

A

increase oxidation

decrease energy storage

134
Q

Higher circulating leptin will stimulate production or (orexogenic/anorexogenic) effectors

A

anorexogenic

135
Q

The inflammatory cytokines released released by resident macrophages in adipose tissue include (3)

A

TNFα
IL6
PAI 1 (plasminogen activating factor)

136
Q

AMPK is activated by (AMP/ATP)

A

AMP

137
Q

AMPK stimulates (catabolism/anabolism)

A

catabolism

138
Q

Adiponectin and leptin (stimulate/inhibit) AMPK

A

stimulate

139
Q

What protein kinase can modulate insulin secretion, fatty acid oxidation and other metabolic rates?

A

AMPK

AMP-activated protein kinase

140
Q

Reduced AMPK activity lead to increased (DAG/insulin) overaccumulation

A

DAG

and insulin resistance

141
Q

Phosphorylated AMPK is (more/less) active

A

more

142
Q

Which is more lipogenic? (fructose/glucose)

A

fructose

143
Q

You are more likely to have T2DM with which type of fat? (Visceral/Subcutaneous)

A

Visceral

144
Q

PPARα ligand agonist that up regulate beta oxidation (Fibrates/Thiazolidinediones)

A

Fibrates

145
Q

PPARγ ligand agonists that promote insulin action and FA uptake (Fibrates/Thiazolidinediones)

A

Thiazolidinediones

146
Q

Obese people have reduced release of (leptin/NEFA/adiponectin/TNFa) from fat cells

A

adiponectin

147
Q

Insulin signaling is inhibited by (PKA/PKC)

A

PKC

148
Q

Name some important antioxidant vitamins:

A
Vitamin E
Vitamin C
Vitamin D
Vitamin A
beta-carotene
149
Q

The three major enzymes for removing free radicals are:

A
  1. SOD
  2. Glutathione peroxidase
  3. Catalase
150
Q

Telomeres are lengthened by a particular enzyme called ______

A

telomerase

151
Q

Decreased growth hormone in mice (extends/shortens) lifespan

A

extends

complicated in humans

152
Q

The derivative of cholesterol that is the precursor for all steroid hormones is ______

A

pregnenolone

153
Q

Glucocorticoids, mineralcorticoids and sex hormones are all derived from _______

A

cholesterol

154
Q

1,25 (OH)2 D3 is also called ______

A

calcitriol

155
Q

Which vitamin is important for vision? including retinol, retinal and retinoic acid

A

Vitamin A

156
Q

Xeropthalmia and hyper keratosis are both characteristics of Vitamin __ deficiency

A

Vitamin A

157
Q

Which vitamin is the precursor of TPP and is critical for the nervous system: prevents Wernicke-Korsakoff syndrome

A

B1: Thiamine

158
Q

Menke’s and Wilsons diseases are both issues with the copper transporting gene. Menkes gene is ______ with copper deficiency. Wilson’s Gene is _______ with copper overload

A

ATP7A

ATP7B

159
Q

Mutations in ATP7b that results in copper overload is called _______ disease

A

Wilson’s disease

160
Q

The three major non-mitochondrial oxidases are:

A
  1. NADPH oxidase
  2. Xanthine oxidase
  3. Monoamine oxidase
161
Q

H2O2 does damage to proteins by damaging adjacent cystine residues forming ______

A

disulfide bonds

162
Q

Metformin is a drug used to phosphorylate and activate _______ to treat T2DM

A

AMPK kinase

163
Q

primary bile acids: (cholic/deoxycholic/lithocholic)

A

cholic

164
Q

A ketone at C-3 of the cyclophenanthrene ring in steroids is most likely found in: (cholesterol ester/steroid hormone)

A

steroid hormone

165
Q

The last common intermediate in the cholesterol, dolichol and ubiquinone biosynthetic pathways is: (acetyl CoA/farnesyl pyrophosphate)

A

farnesyl pyrophosphate

166
Q

When cholesterol levels in the cell are (low/high), S1P protease will be inactive and cleavage of SREBP will not proceed.

A

high

167
Q

where are primary bile acids synthesized

A

liver

168
Q

Biological isoprenoids are precursors to (squalene/ubiquinone/doichol/Vit D)

A

all

169
Q

Synthesis of one molecule of cholesterol utilizes _ (#) molecules of HMG-CoA

A

6

170
Q

synthesis of cholesterol is (anaerobic/aerobic)

A

aerobic

171
Q

Free cholesterol is not the primary storage or transport form of this sterol. Rather it is modified so that it is trapped within carrier lipoproteins or intracellular deposits by: (Conjugation of either glycine or taurine/Acylation at C3)

A

Acylation at C3

172
Q

Patients with sitosterolemia (cannot absorb cholesterol/increased risk of gallstones/hyperabsorb cholesterol and all sterols)

A

hyperabsorb cholesterol and all sterols

173
Q

where does the synthesis of cholesterol take place in the cell (2 organelles)

A

cytosol and ER

174
Q

A decrease in cholesterol-7-hydroxylase (CYP7A1) activity would (decrease/increase) bile acid production

A

decrease

175
Q

A decrease in cholesterol-7-hydroxylase (CYP7A1) activity would (increase/decrease) glycine conjugation

A

no effect

176
Q

A decrease in cholesterol-7-hydroxylase (CYP7A1) activity would (increase/decrease) risk of atherosclerosis

A

increase

177
Q

SREBP is proteolytically cleaved to produce a fragment that (activates/blocks) transcription of the HMG-CoA reductase gene.

A

activates

178
Q

Mevalonate (activates/inhibits) HMG CoA Reductase allosterically and accelerates the rate of enzyme degradation.

A

inhibits

179
Q

statin drugs lead to (decreased/increased) cholesterol flux through bile.

A

increased

180
Q

statin drugs lead to (decreased/increased) circulating HDL cholesterol

A

increased

181
Q

statin drugs lead to (decreased/increased) LDL cell surface receptors

A

increased