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
Apo ___ is an LPL activator, while Apo ____ is an LPL inhibitor
C II | C III
26
HDLs are the only carriers of Apo ____ and Apo ____
A I and A II
27
Tri-Glycerides in VLDL are exchanged with Cholesterol Esters in HDL by the action of _____
CEPT | Cholesterol ester transfer proteins
28
Apo E and Apo C II are put onto other lipoproteins from circulating _____
HDL
29
Excess cholesterol is returned to the liver via ____
HDL
30
Tangier disease is a deficiency in _____ which leads to a lack of HDL particles
ABCA1
31
Scavenger Receptor B1 (SR-B1) helps to clear (VLDL/LDL/HDL/Chylomicrons)
HDL
32
The enzyme that converts cholesterol to cholesterol esters so that it may be stored in HDL is _____
LCAT | Lecithin—cholesterol acyltransferase
33
Cholesterol is moved into the mitochondria for steroid synthesis by the transporter _____
StAR | steroidogenic acute regulatory protein
34
The rate limiting step in the conversion of cholesterol to steroids is catalyzed by the enzyme _____
CYP11A | a cytochrome p450 mixed function oxidase
35
CYP11A is located at the ______
inner mitochondrial membrane
36
Corticotropin releasing hormone (CRH) induces __________ which induces glucocorticoid cortisol secretion
ACTH | Adrenocorticotropic hormone
37
High cortisol concentrations (promotes/inhibits) Corticotropin releasing hormone (CRH)
inhibits (negative feedback)
38
Function of Aldosterone:
Increase Na+ (and H2O) uptake at kidney tubules
39
Angiotensinogen is converted to angiotensin I by ______
Renin
40
Angiotensin I is converted to angiotensin II by ______
ACE (angiotensin converting enzyme)
41
Renin dependent hypertension is treated by ______
ACE inhibitors
42
Steroid hormones are secreted by the (liver/gonads/adrenal cortex/thyroid)
adrenal cortex
43
Hormone that acts on intestines and causes increased calcium absorption is _____
calcitrol
44
The active form of vitamin D in the body is (calcidiol/calcitriol)
calcitriol
45
Calcidiol is converted to calcitriol in the ______
kidneys
46
Calcium levels are monitored by the (organ) ______
parathyroid
47
_____ stimulates intestinal absorption of calcium by binding the Vitamin D receptor (VDR)
calcitrol = 1,25-diOH-D3
48
calcitrol (1,25-diOH-D3) binds the ____ in the cytoplasm of intestinal cells
Vitamin D Receptor (VDR)
49
On an enterocyte, _______ mediates transport of calcium across the apical membrane
Calbindin-D9K
50
Movement of calcium into the epithelial cell is mediated by ______
TRVP5
51
The MEOS consists of a cyt P450 enzyme called ______
CYP2E1
52
In the liver EtOH is converted to Acetaldehyde by _____
ADH | Acetaldehyde deyhydrogenase
53
In the liver Acetaldehyde is converted to Acetate by _____
ALDH | acetaldehyde dehydrogenase
54
The most abundant isozyme of ADH is (ADH1/ADH2/ADH3)
ADH1
55
The isozyme of ADH best for long chain alcohols is (ADH1/ADH2/ADH3)
ADH3
56
(ALDH1/ALDH2) is abundant in the mitochondria
ALDH2
57
(ALDH1/ALDH2) is abundant in the cytoplasm
ALDH1
58
Treating alcoholics with disulfiram inhibits the enzyme ______
ALDH
59
Oxidation of acetylaldehyde occurs in the (organelle) ______
mitochondria
60
The A vitamins have important functions in _____
vision
61
Excess vitamin A accumulates in the (organ) _____
liver (ito cells)
62
Keratomalacia can occur with a deficiency in which vitamin?
Vitamin A
63
Deficiency of Vitamin D can lead to _____ in children and ________ in adults
Rickets | osteomalacia
64
A deficiency in this vitamin results in easy bruising and bleeding
Vitamin K
65
Vitamin K has an essential role in modifying ________, part of the clotting cascade
Prothrombin
66
Which vitamin functions as an antioxidant?
Vitamin E
67
Which vitamin is present in all cell and organelle membranes?
Vitamin E
68
A severe deficiency in this vitamin can lead to impaired collagen formation and scurvy
Vitamin C
69
Synthesis of epinephrin in adrenal glands requires which vitamin?
Vitamin C
70
Which B vitamins are hematopoietic?
B9: Folate B12: Cobalamine
71
Which B vitamins are energy releasing?
B1 Thiamine B2 Riboflavin B3 Niacin B5 Pantothenic Acid B6 Pyridoxine B7 Biotin
72
B1 is ____
Thiamine
73
B2 is ____
Riboflavin
74
B3 is _____
Niacin
75
B5 is _____
Pantothenic acid
76
B6 is _____
Pyridoxine
77
B9 is _____
Folic Acid
78
B12 is _____
Cobalamine
79
A severe deficiency in ____ causes Beri Beri
Thiamine B1
80
Which vitamin is a precursor for NAD and NADP?
Niacin B3
81
Which vitamin is a precursor for FAD and FMN?
Riboflavin B2
82
A deficiency in which vitamin will lead to pellagra?
Niacin
83
Which vitamin is required for synthesis of CoA
Pantothenic acid B5
84
This vitamin is a precursor to for PLP
Pyridoxine B6
85
This vitamin is required for synthesis of GABA and Heme
Pyridoxine B6
86
Which B vitamin is a precursor to tetrahydrafolate?
Folate B9
87
Inadequate levels of vitamin _____ can lead to neural tube defects
B9, Folate
88
Pernicious anemia refers specifically to a deficiency in _____
B12 due to a lack of intrinsic factor
89
Menkes' disease results from an inability to transport _____
Cu
90
One of the earliest symptoms of a zinc deficiency is _____
dermatitis
91
Which mineral is important for insulin to bind its receptor?
chromium
92
Deficiency in this mineral an lead to an enlarged thyroid that becomes a goiter
Iodine
93
Which mineral is a component of glutathione peroxidase?
selenium
94
An overload in in iron can lead to a disease called _______
hemochromatosis
95
Copper is a critical co-factor for (mineral) ______ absorbtion
iron
96
Which vitamin is imperative for blood coagulation?
Vitamin K
97
If intestinal bacteria are missing either due to antibiotics or in new borns, which vitamin may be deficient?
Vitamin K
98
What are the three most common vitamin deficiencies in alcoholics?
Folate B6 Thiamine
99
The "wet brain" associated with B1 deficiency in alcoholics is formally called ______
Wernicke-Korsaoff Syndrome
100
Folate is important for (cell component) _____ synthesis
DNA
101
Most superoxide generation occurs within the ______
mitochondria
102
The most dangerous free radical is ______
*OH free radical
103
Produced by Fenton reaction: | *O2-/*OH-/NO*/H2O2/ONOO-
hydroxyl radical
104
Produced from hydrogen peroxide by ionizing radiation | *O2-/*OH-/NO*/H2O2/ONOO-
hydroxyl radical
105
Produced form the mitochondrial ETC | *O2-/*OH-/NO*/H2O2/ONOO-
Superoxide
106
Produced in the oxidation of hemoglobin | *O2-/*OH-/NO*/H2O2/ONOO-
Superoxide
107
Produced by nitric oxide synthase | *O2-/*OH-/NO*/H2O2/ONOO-
Nitric oxide
108
Produced by the reaction between nitric oxide and superoxide | *O2-/*OH-/NO*/H2O2/ONOO-
ONOO-
109
Produced by myeloperoxidase (MPO) | *O2-/*OH-/NO*/HClO/ONOO-
HClO
110
Substrate for myeloperoxidase | NADP+/NAD+/NADPH/*O2-/H2O2
hydrogen peroxide
111
Product for superoxide dismutase reaction | NADP+/NAD+/NADPH/*O2-/H2O2
hydrogen peroxide
112
Substrate for Glutathione peroxidase | NADP+/NAD+/NADPH/*O2-/H2O2
hydrogen peroxide
113
Product of nitric oxide synthase | NADP+/NAD+/NADPH/*O2-/H2O2
NADP+
114
Reacts with nitric oxide to produce peroxinitrite | NADP+/NAD+/NADPH/*O2-/H2O2
Super oxide
115
Substrate for respiratory burst oxidase | NADP+/NAD+/NADPH/*O2-/H2O2
NADPH
116
Reacts with Fe2+ to produce hydroxyl radical | NADP+/NAD+/NADPH/*O2-/H2O2
H2O2
117
Product of glucose-6-phosphate dehydrogenase reaction | NADP+/NAD+/NADPH/*O2-/H2O2
NADPH
118
Some cases of ALS have been associated with this enzyme | G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione
S.O.D. | Superoxide dismutase
119
Keeps sulfahydryls of proteins reduced | G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione
Reduced Glutathione
120
Chronic granulomatous disease is associated with mutations in this membrane bound complex (G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione)
Nox | NADPH oxidase
121
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)
Glucose-6-phosphate dehydrogenase
122
*O2- is a product of this enzyme | G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione
Nox | NADPH oxidase
123
Decomposes hydrogen peroxide | G-6-P dehydrogenase/S.O.D./Nox/Catalase/Reduced Glutathione
Catalase
124
Reducing protein disulfides | Fe3+/Fe2+/reduced glutathione/oxidized glutathione
reduced glutathione
125
Reacts with H2O2 in Fenton reaction | Fe3+/Fe2+/reduced glutathione/oxidized glutathione
Fe2+
126
Produced by reactions with H2Os in Fenton reaction | Fe3+/Fe2+/reduced glutathione/oxidized glutathione
Fe3+
127
Presenting as the dimeric GSSG | Fe3+/Fe2+/reduced glutathione/oxidized glutathione
oxidized glutathione
128
Leptin (increases/decreases) appetite
decreases
129
The protein UCP1 (uncoupling protein) is found in which type of fat?
brown fat (and beige fat)
130
Which protein in brown fat is responsible for proton leaking across the inner mitochondrial membrane?
UCP1 (uncoupling protein 1)
131
2,4 dinitrophenol (DNP) can function as a ______ to allow protons to leak across the membrane
uncoupler
132
UCP1 (thermogenin) can be activated by which adrenergic receptor?
β3
133
Under normal physiological conditions, KSR2 (kinase suppressor of Ras2) works to (increase/decrease) glucose and fatty acid oxidation
increase oxidation | decrease energy storage
134
Higher circulating leptin will stimulate production or (orexogenic/anorexogenic) effectors
anorexogenic
135
The inflammatory cytokines released released by resident macrophages in adipose tissue include (3)
TNFα IL6 PAI 1 (plasminogen activating factor)
136
AMPK is activated by (AMP/ATP)
AMP
137
AMPK stimulates (catabolism/anabolism)
catabolism
138
Adiponectin and leptin (stimulate/inhibit) AMPK
stimulate
139
What protein kinase can modulate insulin secretion, fatty acid oxidation and other metabolic rates?
AMPK | AMP-activated protein kinase
140
Reduced AMPK activity lead to increased (DAG/insulin) overaccumulation
DAG | and insulin resistance
141
Phosphorylated AMPK is (more/less) active
more
142
Which is more lipogenic? (fructose/glucose)
fructose
143
You are more likely to have T2DM with which type of fat? (Visceral/Subcutaneous)
Visceral
144
PPARα ligand agonist that up regulate beta oxidation (Fibrates/Thiazolidinediones)
Fibrates
145
PPARγ ligand agonists that promote insulin action and FA uptake (Fibrates/Thiazolidinediones)
Thiazolidinediones
146
Obese people have reduced release of (leptin/NEFA/adiponectin/TNFa) from fat cells
adiponectin
147
Insulin signaling is inhibited by (PKA/PKC)
PKC
148
Name some important antioxidant vitamins:
``` Vitamin E Vitamin C Vitamin D Vitamin A beta-carotene ```
149
The three major enzymes for removing free radicals are:
1. SOD 2. Glutathione peroxidase 3. Catalase
150
Telomeres are lengthened by a particular enzyme called ______
telomerase
151
Decreased growth hormone in mice (extends/shortens) lifespan
extends | complicated in humans
152
The derivative of cholesterol that is the precursor for all steroid hormones is ______
pregnenolone
153
Glucocorticoids, mineralcorticoids and sex hormones are all derived from _______
cholesterol
154
1,25 (OH)2 D3 is also called ______
calcitriol
155
Which vitamin is important for vision? including retinol, retinal and retinoic acid
Vitamin A
156
Xeropthalmia and hyper keratosis are both characteristics of Vitamin __ deficiency
Vitamin A
157
Which vitamin is the precursor of TPP and is critical for the nervous system: prevents Wernicke-Korsakoff syndrome
B1: Thiamine
158
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
ATP7A | ATP7B
159
Mutations in ATP7b that results in copper overload is called _______ disease
Wilson's disease
160
The three major non-mitochondrial oxidases are:
1. NADPH oxidase 2. Xanthine oxidase 3. Monoamine oxidase
161
H2O2 does damage to proteins by damaging adjacent cystine residues forming ______
disulfide bonds
162
Metformin is a drug used to phosphorylate and activate _______ to treat T2DM
AMPK kinase
163
primary bile acids: (cholic/deoxycholic/lithocholic)
cholic
164
A ketone at C-3 of the cyclophenanthrene ring in steroids is most likely found in: (cholesterol ester/steroid hormone)
steroid hormone
165
The last common intermediate in the cholesterol, dolichol and ubiquinone biosynthetic pathways is: (acetyl CoA/farnesyl pyrophosphate)
farnesyl pyrophosphate
166
When cholesterol levels in the cell are (low/high), S1P protease will be inactive and cleavage of SREBP will not proceed.
high
167
where are primary bile acids synthesized
liver
168
Biological isoprenoids are precursors to (squalene/ubiquinone/doichol/Vit D)
all
169
Synthesis of one molecule of cholesterol utilizes _ (#) molecules of HMG-CoA
6
170
synthesis of cholesterol is (anaerobic/aerobic)
aerobic
171
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)
Acylation at C3
172
Patients with sitosterolemia (cannot absorb cholesterol/increased risk of gallstones/hyperabsorb cholesterol and all sterols)
hyperabsorb cholesterol and all sterols
173
where does the synthesis of cholesterol take place in the cell (2 organelles)
cytosol and ER
174
A decrease in cholesterol-7-hydroxylase (CYP7A1) activity would (decrease/increase) bile acid production
decrease
175
A decrease in cholesterol-7-hydroxylase (CYP7A1) activity would (increase/decrease) glycine conjugation
no effect
176
A decrease in cholesterol-7-hydroxylase (CYP7A1) activity would (increase/decrease) risk of atherosclerosis
increase
177
SREBP is proteolytically cleaved to produce a fragment that (activates/blocks) transcription of the HMG-CoA reductase gene.
activates
178
Mevalonate (activates/inhibits) HMG CoA Reductase allosterically and accelerates the rate of enzyme degradation.
inhibits
179
statin drugs lead to (decreased/increased) cholesterol flux through bile.
increased
180
statin drugs lead to (decreased/increased) circulating HDL cholesterol
increased
181
statin drugs lead to (decreased/increased) LDL cell surface receptors
increased