Biochem Flashcards
(500 cards)
1
Q
2 carbons of the purine nitrogenous bases are derived from molecule(s)?
A
THF; Glycine\
2
Q
2 nitrogens of the purine nitrogenous bases are derived from which amino acid?
A
Glutamine\
3
Q
A defect in what enzyme in Tyrosine metabolism causes Albinism?
A
Tyrosinase\
4
Q
A thiamine deficiency will impair which enzyme in the HMP shunt?
A
Transketolase\
5
Q
Acetyl-CoA activates which enzyme in gluconeogenesis?
A
Pyruvate Carboxylase\
6
Q
Aconitase forms which carbon structure in the TCA cycle?
A
Isocitrate\
7
Q
Administration of which antimicrobial can cause a Niacin deficiency?
A
Isoniazid\
8
Q
Alanine inhibits which enzyme in glycolysis?
A
Pyruvate kinase\
9
Q
All-trans-retinoic acid (ATRA) is used a treatment for which condition?
A
Acute Promyelocytic Leukemia\
10
Q
Alpha-Ketoglutarate Dehydrogenase forms which carbon structure in the TCA cycle?
A
Succinyl-CoA\
11
Q
An overdose of which over-the-counter drug acts as an uncoupling agent of the inner mitochondrial membrane?
A
Aspirin\
12
Q
At what age does Abetalipoproteinemia present?
A
Infancy\
13
Q
At what stage does Classic Galactosemia present?
A
When started on breast milk\
14
Q
At what stage does Hereditary Fructose Intolerance present?
A
When weaned from breast milk\
15
Q
Azathioprine and 6-Mercaptopurine are activated by which enzyme?
A
HGPRT\
16
Q
Azathioprine and 6-MP is metabolized by which enzyme?
A
Xanthine Oxidase\
17
Q
Bacteria methylate which residue(s) on their DNA?
A
Adenine; Cytosine\
18
Q
Biotin is a cofactor for which enzymes?
A
Pyruvate carboxylase; Acetyl-CoA-carboxylase; Propionyl-CoA carboxylase\
19
Q
Biotin is a cofactor for which enzymes?
A
Pyruvate carboxylase; Acetyl-CoA-carboxylase; Propionyl-CoA carboxylase\
20
Q
\f0 HDL?
A
Upregulation of PPAR-alpha\
21
Q
Can the liver use ketone bodies for fuel?
A
No\
22
Q
Cholesteryl esters are combinations of which molecules?
A
Cholesterol; Fatty acid\
23
Q
Chronic excess of Vitamin A can cause enlargement of which organ?
A
Liver\
24
Q
Citrate inhibits which enzyme in glycolysis?
A
PFK-1\
25
Citrate inhibits which enzyme in glycolysis?
PFK-1\
26
Citrate Synthase forms which carbon structure in the TCA cycle?
Citrate\
27
Complex I passes electrons to which molecule?
Coenzyme Q\
28
Complex III passes electrons to which molecule?
Cytochrome C\
29
Corneal vascularization is a symptom of which vitamin deficiency?
Vitamin B2 (Riboflavin)\
30
Creatinine is derived from which molecule?
Creatine\
31
Defect in Lipoprotein Lipase can cause which familial dyslipidemia?
Hyperchylomicronemia\
32
Deficiency in which enzyme in glycolysis can result in extravascular hemolysis of RBCs?
Pyruvate Kinase Deficiency\
33
Deficiency in which vitamin can cause a hemolytic anemia?
Vitamin E\
34
Deficiency in which vitamin can cause burning feet?
Vitamin B5\
35
Deficiency in which vitamin can cause Cheilosis?
Vitamin B2 (Riboflavin)\
36
Deficiency in which vitamin can cause tetany?
Vitamin D\
37
Diphyllobothrium latum causes a deficiency in which Vitamin?
Vitamin B12\
38
DNA methylation methylates which nitrogenous base(s)?
Cytosine Adenine\
39
Does Marasmus cause edema?
No\
40
Does Tay-Sachs disease have hepatosplenomegaly?
No\
41
Excess levels of which metabolite triggers production of ketone bodies?
Acetyl-CoA\
42
Ezetimibe blocks the absorption of which molecule in the small intestine?
Cholesterol\
43
F16BP activates which enzyme in glycolysis?
Pyruvate kinase\
44
FAD+ is derived from which Vitamin?
Vitamin B2 (Riboflavin)\
45
Foam cells are seen in which Lyosomal storage disease?
Niemann-Pick disease\
46
Folate is found in which type of foods?
Leafy green vegetables\
47
For which enzymes is thiamine a cofactor?
Pyruvate dehydrogenase; alpha-ketoglutarate dehydrogenase; alpha-ketoacid dehydrogenase; transketolase\
48
From which metabolic process is Ribose 5-phosphate formed which is used in purine synthesis?
HMP Shunt\
49
Fructose and glucose together make which disaccharide?
Sucrose\
50
Fructose is metabolized to which molecule, which can enter glycolysis?
Glyceraldehyde-3-phosphate\
51
Fumarase forms which carbon structure in the TCA cycle?
Malate\
52
GABA is a derivative of which amino acid?
Glutamate\
53
Galactokinase deficiency can cause which developmental defects?
Failure to track objects Lack of social smile, relating to vision loss due to cataracts\
54
Galactose and glucose together make which disaccharide?
Lactose\
55
Garlic-breath describes what type of poisoning?
Arsenic\
56
Globoid cells are seen in which lysosomal storage disease?
Krabbe disease\
57
Glycerol is converted to Glycerol-3-phosphate by what enzyme?
Glycerol Kinase\
58
Glycogen Phosphorylase releases which molecule?
Glucose-1-phosphate\
59
HDL is a repository for which apolipoproteins?
Apo C-II; Apo E\
60
High levels of Acetyl-CoA in the mitochondria drive which enzyme to metabolize Pyruvate?
Pyruvate Carboxylase\
61
High levels of orotic acid in the urine with hyperammonemia indicates which disease?
OTC deficiency\
62
Histones are rich in which amino acids?
Lysine; Arginine\
63
Homocysteine combines with which amino acid to create Cystathionine?
Serine\
64
Hormone sensitive lipase is activated by which hormone(s)?
Glucagon; Epinephrine\
65
\f0 TG?
Upregulation of Lipoprotein lipase\
66
\f0 TG?
Upregulate Lipoprotein Lipase\
67
How do Fibrates cause gallstones?
Inactivating 7alpha-hydroxylase\
68
How does Arsenic inhibit the Pyruvate Dehydrogenase complex?
Inhibits Lipoic Acid\
69
How does hyperammonemia inhibit the TCA cycle?
Depletion of alpha-KG\
70
How does Lactulose prevent absorption of NH4?
Lowers colonic pH and this promotes NH4+ formation over NH3, trapping it in the colon\
71
How does Vitamin C aid in the absorption of iron?
Conversion of Fe3+ to Fe2+\
72
Hunter syndrome involves a deficiency in which enzyme?
Iduronate-2-sulfatase\
73
Hurler syndrome involves a deficiency in which enzyme?
Alpha-L-iduronidase\
74
Hypohydrosis indicates which lysosomal storage disease?
Fabry disease\
75
Hypoxanthine is the nitrogenous base of which nucleotide?
IMP\
76
In amino acid breakdown, the amino group is initially transferred to which molecule in the muscle?
Alpha-ketoglutarate\
77
In Carcinoid Syndrome, there is a deficiency in which Vitamin?
Vitamin B3 (Niacin)\
78
In Cystinuria, there is decreased reabsorption of what molecules in the proximal renal tubule?
Cystine; Ornithine; Lysine; Arginine\
79
In Diabetic Ketoacidosis, low levels of which substance of the TCA cycle causes it to stall?
Oxaloacetate\
80
In Essential Fructosuria, which enzyme allows Fructose to enter glycolysis?
Hexokinase\
81
In fatty acid synthesis, which enzyme converts Citrate into Acetyl-CoA?
ATP-Citrate Lyase\
82
In gluconeogensis, where in the cell does FBPase-1 function?
Cytosol\
83
In gluconeogensis, where in the cell does Glucose-6-phosphatase function?
Endoplasmic reticulum\
84
In gluconeogensis, where in the cell does PEP Carboxykinase function?
Cytosol\
85
In gluconeogensis, where in the cell does Pyruvate Carboxylase function?
Mitochondria\
86
In glycogen hormonal regulation, what enzymes does glucagon phosphorylate?
Glycogen phosphorylase; Glycogen synthase\
87
In histone acetylation, an acetyl group is added to which amino acid residue?
Lysine\
88
In MCAD deficiency, what builds up in the blood?
Fatty acyl carnitine\
89
In pyrimidine synthesis, which amino acid is required for Carbamoyl Phosphate synthesis?
Glutamine\
90
In pyrimidine synthesis, which amino acid is required for Orotic Acid synthesis?
Aspartate\
91
In terms of cholesterol absorption, ACAT is found in which cell types?
Enterocytes; Hepatocytes\
92
In the kidney, URAT1 causes the absorption of which substance into the tubular cells?
Uric acid\
93
In the kidney, URAT1 causes the secretion of which substance into the tubular lumen?
Organic anions (such as Lactate)\
94
In the Malate shuttle for NADH entry, via which intermediate does Oxaloacetate leave the mitochondria?
Aspartate\
95
In the Malate shuttle, what does Oxaloacetate get converted to in order to leave the mitochondria?
Aspartate\
96
In what organelle is Glucose-6-Phosphatase active?
Endoplasmic Reticulum\
97
In which amino acid metabolism disorder must patients avoid Aspartame?
Phenylketonuria (PKU)\
98
In which conditions are there high levels of orotic acid in the urine?
Orotic aciduria; OTC deficiency\
99
In which familial dyslipidemia can patients get an MI before age 20?
Familial hypercholesterolemia\
100
In which Lysosomal Storage disease is there an accumulation of Ceramide Trihexose?
Fabry disease\
101
In which Lysosomal Storage disease is there an accumulation of Cerebroside sulfate?
Metachromatic leukodystrophy\
102
In which Lysosomal Storage disease is there an accumulation of Galactocerebroside?
Krabbe disease\
103
In which Lysosomal Storage disease is there an accumulation of Glucocerebroside?
Gaucher disease\
104
In which Lysosomal Storage disease is there an accumulation of GM2?
Tay-Sachs disease\
105
In which Lysosomal Storage disease is there an accumulation of Sphingomyelin?
Niemann-Pick disease\
106
In which Lysosomal Storage Diseases are there cherry red spots on the macula?
Tay Sachs disease; Niemann-Pick disease\
107
In which pulmonary condition can activation of Vitamin D occur independent of PCT cells in the kidney?
Sarcoidosis\
108
Is gluconeogenesis intact in Cori disease?
Yes\
109
Isocitrate Dehydrogenase forms which carbon structure in the TCA cycle?
Alpha-Ketoglutarate\
110
Ketone bodies are used as a source of energy by which organs?
Muscle; Heart; Brain\
111
Lack of which enzyme in purine breakdown can cause SCID?
Adenosine Deaminase\
112
Lactose is a combination of which two monosaccharides?
Galactose; Glucose\
113
LDH can be used to differentiate between which types of pleural effusions?
Transudate Exudate\
114
Lipoprotein lipase metabolizes which molecule from chylomicrons?
Triglycerides\
115
Lipoprotein Lipase requires which lipoprotein for activation?
Apo C-II\
116
Low levels of Acetyl-CoA in the mitochondria drive which enzyme to metabolize Pyruvate?
Pyruvate Dehydrogenase\
117
Lyosomes with onion skinning are seen in which lysosomal storage disease?
Tay-Sachs disease\
118
Malate Dehydrogenase forms which carbon structure in the TCA cycle?
Oxaloacetate\
119
MAO breaks down which molecule, which is then excreted as 5-HIAA?
Serotonin\
120
Massive consumption of raw egg whites can cause what vitamin deficiency?
Vitamin B7 (biotin)\
121
Melanin is derived from which amino acid?
Tyrosine\
122
Melatonin is derived from which amino acid?
Tryptophan\
123
Melatonin is synthesized from which neurotransmitter?
Serotonin\
124
Metabolism of the branched chain amino acids depends on which enzyme?
Alpha-ketoacid dehydrogenase\
125
Metyrosine inhibits which enzyme in catecholamine synthesis?
Tyrosine Hydroxylase\
126
Myopathy with Statins is increased when taking what other drugs?
Fibrates; Niacin\
127
N-acetylglutamate activates which enzyme in the Urea cycle?
CPS I\
128
Niacin can be synthesized by the body from which amino acid?
Tryptophan\
129
Niacin inhibits which enzyme in adipocytes?
Hormone sensitive lipase\
130
Niacin is derived from which amino acid?
Tryptophan\
131
Night blindness is a symptom of which vitamin deficiency?
Vitamin A\
132
Nitric Oxide Synthase uses which amino acid to create Nitric Oxide?
Arginine\
133
On which lipoproteins is C-II found?
Chylomicrons; VLDL; HDL\
134
On which molecule(s) is Apo B-48 found?
Chylomicrons; Chylomicron remnants\
135
Patients taking Levodopa/Carbidopa should avoid what Vitamin, in order to avoid the systemic effects of Dopamine?
Vitamin B6\
136
Patients with Classic Galactosemia must exclude what from their diet?
Galactose; Lactose\
137
Patients with Hartnup disease have a deficiency in which Vitamin?
Vitamin B3 (Niacin)\
138
Patients with Hereditary Fructose Intolerance will have a build-up of which molecule in Fructose metabolism?
Fructose-1-Phosphate\
139
Phenylketonuria (PKU) most commonly involves a deficiency in which enzyme?
Phenylalanine hydroxylase\
140
Pyruvate is transported into the mitochondria for which cellular processes?
TCA cycle; Gluconeogenesis (Pyruvate Carboxylase is in the mitochondria)\
141
SAM is synthesized from which amino acid?
Methionine\
142
SAM that loses Adenosine and a Methyl group becomes which molecule?
Homocysteine\
143
Statins increase the number of which type of receptor in the liver?
LDL receptors\
144
Subacute Combined Degeneration involves a deficiency in which Vitamin(s)?
Vitamin B12\
145
Succinate Dehydrogenase forms which carbon structure in the TCA cycle?
Fumarate\
146
Succinyl-CoA Synthetase forms which carbon structure in the TCA cycle?
Succinate\
147
Sucrose is a combination of which two monosaccharides?
Fructose; Glucose\
148
Synthesis of Niacin from its amino acid precursor requires which cofactor(s)?
Vitamin B2; Vitamin B6\
149
The carbons and nitrogens in Urea are derived from which molecules?
NH3; CO2; Aspartate\
150
The liver secretes which lipoprotein(s) to deliver cholesterol and triglycerides to the body?
VLDL; HDL\
151
The Pyruvate Dehydrogenase complex is located in which part of the cell?
Mitochondria\
152
The Pyruvate Dehydrogenase complex requires what cofactors?
Thiamine pyrophosphate (B1); FAD (B2); NAD (B3); Coenzyme A (B5); Lipoic acid\
153
Thiamine deficiency causes which type of cardiomyopathy?
Dilated cardiomyopathy\
154
Thiamine is a cofactor for which enzyme of the HMP shunt?
Transketolase\
155
Thiamine is a cofactor for which enzyme(s) of the TCA cycle?
Alpha-ketoglutarate dehydrogenase\
156
Thiamine is a cofactor for which enzymes?
Pyruvate dehydrogenase; Alpha-ketoglutarate dehydrogenase; alpha-ketoacid dehydrogenase; Transketolase\
157
Thyroid hormone is derived from which amino acid?
Tyrosine\
158
Treatment of Maple Syrup Urine Disease involve supplementation with which vitamin?
Thiamine\
159
Tyrosine is degraded into what component of the TCA cycle?
Fumarate\
160
UMP inhibits what enzyme in pyrimidine synthesis?
Carbamoyl Phosphate Synthetase II\
161
Uridine is the treatment for what defect in pyrimidine synthesis?
Orotic aciduria\
162
Uridine is the treatment for which disease of Pyrimidine Synthesis?
Orotic Aciduria\
163
Vitamin A is used to treat which infectious disease?
Measles\
164
Vitamin B12 contains which metal element?
Cobalt\
165
Vitamin C is an ancillary treatment for which hemoglobin modification condition?
Methemoglobinemia\
166
What accumulates in Hunter syndrome?
Heparan sulfate; Dermatan sulfate\
167
What accumulates in Hurler syndrome?
Heparan sulfate; Dermatan sulfate\
168
What activates Fructose-1,6-bisphosphatase?
Citrate\
169
What activates Isocitrate Dehydrogenase?
ADP\
170
What activates Pyruvate Kinase?
Fructose 1,6 BP\
171
What aids the absorption of Iron via conversion of Fe3+ to Fe2+?
Vitamin C\
172
What anti-malarial drugs can precipitate a G6PD deficiency crisis?
Primaquine Quinidine\
173
What apolipoprotein is required on Chylomicrons for secretion from enterocytes?
Apo B-48\
174
What apolipoproteins are found on VLDL?
Apo B-100; Apo C-II; Apo E\
175
What apolipoproteins are present on Chylomicron remnants?
E; B-48\
176
What apolipoproteins are present on Chylomicrons?
B-48; C-II; E\
177
What apolipoproteins are present on HDL?
A-I; C-II; E\
178
What apolipoproteins are present on IDL?
E; B-100\
179
What apolipoproteins are present on LDL?
B-100\
180
What apolipoproteins are present on VLDL?
B-100; C-II; E\
181
What apoproteins are on HDL?
A-I; C-II; Apo E\
182
What are 3 uncoupling agents of oxidative phosphorylation?
2,4, DNP; Aspirin; Thermogenin in brown fat\
183
What are examples of purine rich foods, which are more likely to precipitate an acute gout attack?
Red meat; Seafood\
184
What are the acidic amino acids?
Aspartic acid; Glutaminc acid\
185
What are the activators of Glycogen synthase?
Glucose-6-phosphate; Insulin; Cortisol\
186
What are the activators of PFK-1?
AMP; Fructose-2,6-bisphosphate\
187
What are the adverse effects of bile acid resins?
GI upset; Decreased absorption of fat soluble vitamins/drugs\
188
What are the adverse effects of Fibrates?
Myopathy; Cholesterol gallstones\
189
What are the adverse effects of HMG-CoA reductase inhibitors?
Hepatotoxicity; Myopathy\
190
What are the adverse effects of Niacin?
Facial flushing; Hyperglycemia; Hyperuricemia\
191
What are the antioxidant vitamins?
Vitamin A; Vitamin C; Vitamin E\
192
What are the basic amino acids?
Histidine; Lysine; Arginine\
193
What are the branched chain amino acids?
Leucine; Isoleucine; Valine\
194
What are the branched chain amino acids?
Leucine; Isoleucine; Valine\
195
What are the cardiac findings in Carcinoid Syndrome?
Right-sided valvular heart disease\
196
What are the cardiovascular effects of Homocystinuria?
Thrombosis/Atherosclerosis Stroke/MI\
197
What are the classic patients who have a folate deficiency?
Alcoholics; Pregnant women\
198
What are the clinical findings in Pyruvate dehydrogenase complex deficiency?
Neurologic defects; Lactic acidosis;
199
What are the clinical findings in the skin of patients with Vitamin A deficiency?
Dry, scaly skin (xerosis cutis)\
200
What are the clinical indications for Hydroxyurea?
Sickle cell anemia; Myeloproliferative disorders\
201
What are the components of a nucleotide?
Phosphate group; Nitrogenous base; Sugar\
202
What are the dietary sources of Vitamin A?
Leafy vegetables; Liver\
203
What are the histone proteins in the nucleosome core?
H2A H2B; H3; H4\
204
What are the inducers of PFK-1?
AMP; Fructose 2,6 bisphosphate\
205
What are the inhibitors of Glycogen phosphorylase?
Glucose-6-phosphate; Insulin; ATP\
206
What are the inhibitors of Glycogen synthase?
Epinephrine; Glucagon\
207
What are the inhibitors of PFK-1?
ATP; Citrate\
208
What are the inhibitors of PFK-1?
ATP; Citrate\
209
What are the late symptoms of Fabry disease?
Renal failure; Cardiovascular disease\
210
What are the peripheral blood smear findings in G6PD deficiency?
Heinz bodies; Bite cells\
211
What are the Phenyl ketones?
Phenylpyruvate; Phenyllacetate; Phenyllactate\
212
What are the presenting symptoms of Abetalipoproteinemia?
Fat malabsorption; Steatorrhea; Failure to thrive\
213
What are the products formed from Pyruvate Dehydrogenase?
Acetyl-CoA; NADH; CO2\
214
What are the purely ketogenic amino acids?
Leucine; Lysine\
215
What are the side effects of an excess of Niacin?
Facial flushing Hyperglycemia; Hyperuricemia\
216
What are the sources of nitrogens in urea?
NH3; Aspartate\
217
What are the stimulators of Glycogen phosphorylase?
Epinephrine; Glucagon; AMP\
218
What are the symptoms of acute Vitamin A toxicity?
Nausea; Vomiting; Vertigo; Blurred vision\
219
What are the symptoms of Biotin deficiency?
Dermatitis; Enteritis; Alopecia\
220
What are the symptoms of dry beriberi?
Polyneuritis; Symmetrical muscle wasting\
221
What are the symptoms of Dry beriberi?
Polyneuropathy; Symmetrical muscle wasting\
222
What are the symptoms of Hartnup disease?
Similar to Pellagra\
223
What are the symptoms of Pellagra?
Dermatitis; Diarrhea; Dementia; Death\
224
What are the symptoms of riboflavin deficiency?
Chelosis; Corneal vascularization\
225
What are the symptoms of Systemic Primary Carnitine Deficiency?
Muscle weakness; Hypotonia; Hypoketotic hypoglycemia\
226
What are the symptoms of Vitamin B5 deficiency?
Dermatitis; Enteritis; Alopecia; Adrenal insufficiency\
227
What are the symptoms of wet beriberi?
Tachycardia; High output heart failure; Edema\
228
What are the symptoms of Wet beriberi?
Tachycardia; High-ouput heart failure; Edema\
229
What are the Vitamin K dependent clotting factors?
II, VII, IX, X, Protein C, and Protein S\
230
What are two disorders of thiamine deficiency?
Beriberi; Wernicke-Korsakoff syndrome\
231
What artifical sweetener must be avoided in patients with Phenylketonuria?
Aspartame as it contains phenylalanine\
232
What can be used to blunt the facial flushing of Niacin Excess?
Aspirin\
233
What causes Hypoglycemia after feeding in Hereditary Fructose Intolerance?
Depletion of ATP\
234
What causes loss of gluconeogenesis in Hereditary Fructose Intolerance?
Depletion of ATP\
235
What cell type are Gaucher cells?
Macrophages\
236
What cell type is characteristic in Krabbe disease?
Globoid cells\
237
What cells are most effected by Pyruvate Kinase Deficiency?
RBCs\
238
What cellular pathway is the origin of NADPH used in the respiratory burst?
HMP Shunt\
239
What clinical syndromes develop from Thiamine (Vitamin B1) deficiency?
Beriberi; Wernicke-Korsakoff syndrome\
240
What co-factors are required for the Pyruvate Dehydrogenase Complex?
Thiamine pyrophosphate; FAD; NAD; Coenzyme A (CoA); Lipoic acid\
241
What cofactor for the Pyruvate Dehydrogenase Complex is a derivative of niacin?
NAD+\
242
What cofactor for the Pyruvate Dehydrogenase Complex is a derivative of panthothenic acid?
Coenzyme A\
243
What cofactor for the Pyruvate Dehydrogenase Complex is a derivative of riboflavin?
FAD+\
244
What cofactor is required for Glycogen Phosphorylase?
Vitamin B6\
245
What cofactor(s) is(are) required by PEP Carboxykinase?
GTP\
246
What cofactor(s) is(are) required by Pyruvate Carboxylase?
ATP; Biotin; CO2\
247
What cofactors are required to convert Tryptophan to Niacin?
Vitamin B2; Vitamin B6\
248
What condition affecting the curvature of the spine is seen in patients with Homocystinuria?
Kyphosis\
249
What direction does the lens of the eye sublux in Homocystinuria?
Down and in\
250
What direction does the lens of the eye sublux in Marfan syndrome?
Up and out\
251
What does a nucleosome consist of?
Histone DNA\
252
What does Beta-oxidation produce?
NADH; FADH2; Acetyl-CoA\
253
What does Glycogen Phosphorylase produce when it breaks off a monomer from glycogen?
Glucose-1-phosphate\
254
What does NADH bind to, in order to enter the mitochondria in the Malate Shuttle?
Oxaloacetate\
255
What does the brain use for energy for metabolism?
Glucose; Ketones\
256
What energy storage molecule is produced through the action of G6PD?
NADPH\
257
What enzyme catalyzes the first step in Beta-oxidation?
Acyl-CoA dehydrogenase\
258
What enzyme catalyzes the rate limiting step of fatty acid synthesis?
Acetyl-CoA Carboxylase\
259
What enzyme catalyzes the rate limiting step of the TCA cycle?
Isocitrate Dehydrogenase\
260
What enzyme catalyzyes the rate limiting step in the Urea cycle?
Carbamoyl Phosphate Synthetase I\
261
What enzyme commits glucose to glycolysis?
PFK-1\
262
What enzyme converts glucose-6-phosphate into 6 phosphogluconolactone?
G6PD\
263
What enzyme converts OAA to PEP in gluconeogenesis?
PEP Carboxykinase\
264
What enzyme converts Pyruvate to OAA in gluconeogenesis?
Pyruvate Carboxylase\
265
What enzyme in the HMP Shunt requires thiamine as a cofactor?
Transketolase\
266
What enzyme is deficienct in Krabbe disease?
Galactocerebrosidase\
267
What enzyme is deficient in Fabry disease?
alpha-galactosidase A\
268
What enzyme is deficient in Gaucher disease?
Glucocerebrosidase\
269
What enzyme is deficient in Metachromatic leukodystrophy?
Arylsulfatase A\
270
What enzyme is deficient in Niemann-Pick disease?
Sphingomyelinase\
271
What enzyme is deficient in Tay-Sachs disease?
Hexosaminidase A\
272
What enzyme is responsible for the rate limiting step of glycolysis?
PFK-1\
273
What enzyme is used to resynthesize BH4 from BH2?
Dihydropteridine Reductase\
274
What enzyme is used to synthesize HMG-CoA?
HMG-CoA Synthase\
275
What enzyme metabolizes Azathioprine?
Xanthine Oxidase\
276
What enzyme of fatty acid synthesis is activated by Citrate?
Acetyl-CoA carboxylase\
277
What enzyme of glycolysis is inhibited by Citrate?
PFK-1\
278
What enzyme performs the rate limiting step in the Oxidative part of the HMP Shunt?
G6PD\
279
What enzyme reactions require biotin as a cofactor?
Pyruvate Carboxylase; Acetyl-CoA Carboxylase; Propionyl-CoA Carboxylase\
280
What enzyme(s) convert Dopamine to Homovanillic Acid (HVA)?
MAO; COMT\
281
What ethnicity has a higher prevalence of G6PD Deficiency?
African Americans\
282
What food is a classic cause of oxidative damage in patients with G6PD deficiency?
Fava beans\
283
What gene is mutated in Abetalipoproteinemia?
Microsomal Triglyceride Transfer Protein (MTTP)\
284
What histone protein is not in the histone core?
H1\
285
What immunodeficiency syndrome can cause albinism?
Chediak-Higashi Syndrome\
286
What inhibits Acetyl-CoA Carboxylase?
Glucagon Palmitoyl-CoA\
287
What inhibits alpha-Ketoglutarate Dehydrogenase?
Succinyl-CoA; NADH; ATP\
288
What inhibits Fructose-1,6-bisphosphatase?
AMP Fructose-2,6-bisphosphate\
289
What inhibits Glutamine-PRPP-amidotransferase?
High levels of IMP, AMP, GMP\
290
What inhibits Isocitrate Dehydrogenase?
ATP NADH\
291
What inhibits Pyruvate Kinase?
ATP; Alanine\
292
What inhibits the rate limiting step in Fatty acid oxidation?
Malonyl-CoA\
293
What is a component of the electron transport chain that is derived from Riboflavin?
FMN\
294
What is a hematological consequence of Gaucher disease?
Pancytopenia\
295
What is a musculoskeletal effect of chronic Vitamin A excess?
Arthralgias\
296
What is a reproductive side effect of Zinc deficiency?
Hypogonadism\
297
What is a skin finding of hyperlipidemia?
Xanthomas\
298
What is a skin lesioning finding of early Fabry disease?
Angiokeratomas\
299
What is added to Galactose-1-P to create Glucose-1-P?
UDP Glucose\
300
What is an allosteric activator of Carbamoyl Phosphate Synthetase I?
N-acetylglutamate\
301
What is an allosteric activator of Pyruvate Carboxylase?
Acetyl-CoA\
302
What is an ocular condition associated with Abetalipoproteinemia?
Retinitis pigmentosa\
303
What is an ocular finding in Hurler syndrome?
Corneal clouding\
304
What is an ocular finding in Niemann-Pick disease?
Cherry-red spot on macula\
305
What is an ocular finding of I-cell disease?
Corneal clouding\
306
What is combined to form Palmitate?
Acetyl CoA; Malonyl CoA\
307
What is deficient in Hyperchylomicronemia?
Lipoprotein Lipase; Apo C-II\
308
What is deficient in patients with Abetalipoproteinemia?
B-48; B-100\
309
What is normal plasma pH?
7.35 - 7.45\
310
What is packaged inside of the Chylomicron?
Triglycerides; Cholesteryl Esters; Vitamins A, E, K, D\
311
What is produced at the end of the oxidative portion of the HMP shunt?
Ribulose-5 phosphate; 2 molecules of NADPH; CO2\
312
What is produced when Acetyl-CoA levels exceed the capacity of the TCA cycle?
Ketone bodies\
313
What is the accumulated substance in Fabry disease?
Ceramide trihexoside\
314
What is the accumulated substance in Gaucher disease?
Glucocerebroside\
315
What is the accumulated substance in Krabbe disease?
Galactocerebroside and Psychosine\
316
What is the accumulated substance in Metachromatic leukodystrophy?
Cerebroside sulfate\
317
What is the accumulated substance in Niemann-Pick disease?
Sphingomyelin\
318
What is the accumulated substance in Tay-Sachs disease?
GM2\
319
What is the affect of AMP on Glycogen synthase/phosphorylase?
Stimulate Glycogen Phosphorylase\
320
What is the affect of ATP on Glycogen synthase/phosphorylase?
Inhibit Glycogen Phosphorylase\
321
What is the affect of Cortisol on Glycogen synthase/phosphorylase?
Stimulate Glycogen Synthase\
322
What is the antidote for overdoses of ethylene glycol or methanol?
Fomepizol; Ethanol; Dialysis\
323
What is the breakdown product of normetanephrine (from NE) and metanephrine (from EPI)?
Vanillylmandelic acid (VMA)\
324
What is the cardiac finding in a baby born to a mother with Maternal PKU?
Congenital heart defects\
325
What is the classic finding of Megaloblastic Anemias on a peripheral blood smear?
Hypersegmented neutrophils\
326
What is the classic pathologic finding in Tay-Sach disease?
Onion skinning of lysosomes\
327
What is the classic triad of symptoms in Wernicke-Korsakoff syndrome?
Confusion; Ophthalmoplegia; Ataxia\
328
What is the clinical presentation of Essential Fructosuria?
Fructose in the blood and urine\
329
What is the cofactor for AST and ALT?
Vitamin B6\
330
What is the cofactor for DOPA Decarboxylase?
Vitamin B6\
331
What is the cofactor for Dopamine-Beta-Hydroxylase?
Vitamin C\
332
What is the cofactor for Glycogen Phosphorylase?
Vitamin B6\
333
What is the cofactor for Methionine Synthase?
Vitamin B12\
334
What is the cofactor for Methionine Synthase?
Vitamin B12\
335
What is the cofactor for Methylmalonyl-CoA mutase?
Vitamin B12\
336
What is the cofactor for Phenylalanine Hydroxylase?
BH4\
337
What is the cofactor for Phenylethanolamine-N-methyltransferase?
SAM\
338
What is the cofactor for Tyrosine Hydroxylase?
BH4\
339
What is the cofactor requried by the enzyme that converts Dopamine to Norepinephrine?
Vitamin C\
340
What is the color of fresh urine in patients with Alkaptonuria?
Normal\
341
What is the color of urine in patients with Alkaptonuria that has been left exposed to air?
Black\
342
What is the dietary treatment for Hartnup disease?
High protein diet; Vitamin B3 (Niacin)\
343
What is the dominant source of glucose in between meals?
Hepatic glycogenolysis\
344
What is the effect of activating PPAR-alpha by Fibrates?
Increased synthesis of HDL\
345
What is the effect of Niacin on adipocytes as a lipid-drug?
Inhibits hormone sensitive lipase\
346
What is the effect of Niacin on the liver as lipid-drug?
Reduces hepatic production of VLDL\
347
What is the form of cholesterol in chylomicrons?
Cholesteryl Ester\
348
What is the function of Apo A-I?
LCAT activation\
349
What is the function of Apo B-48?
Mediates chylomicron secretion into lymphatics from enterocytes\
350
What is the function of Apo B-100?
Binds LDL receptor\
351
What is the function of Apo C-II?
Lipoprotein Lipase activator\
352
What is the function of Apo E?
Mediates reuptake of lipoproteins by the liver\
353
What is the function of LCAT in HDL?
Esterifies cholesterol\
354
What is the genetic inheritance of Abetalipoproteinemia?
Autosomal recessive\
355
What is the genetic inheritance of Alkaptonuria?
Autosomal recessive\
356
What is the genetic inheritance of Fabry disease?
X-linked recessive\
357
What is the genetic inheritance of Familial Hypercholesterolemia?
Autosomal Dominant\
358
What is the genetic inheritance of G6PD deficiency?
X-linked recessive\
359
What is the genetic inheritance of Glycogen Storage Diseases types I, II, III, and V?
Autosomal recessive\
360
What is the genetic inheritance of Hartnup Disease?
Autosomal recessive\
361
What is the genetic inheritance of Hunter Syndrome?
X-linked recessive\
362
What is the genetic inheritance of Hurler Syndrome?
Autosomal recessive\
363
What is the genetic inheritance of Hypertriglyceridemia?
Autosomal Dominant\
364
What is the genetic inheritance of Lesch-Nyhan syndrome?
X-linked recessive\
365
What is the genetic inheritance of Maple Syrup Urine Disease?
Autosomal recessive\
366
What is the genetic inheritance of Orotic aciduria?
Autosomal recessive\
367
What is the genetic inheritance of OTC deficiency?
X-linked recessive\
368
What is the genetic inheritance of PDH Complex Deficiency?
X-linked recessive\
369
What is the genetic inheritance of Phenylketonuria?
Autosomal recessive\
370
What is the hematologic consequence of Kwashiokor?
Anemia\
371
What is the intermediate in the conversion of Pyruvate to PEP in gluconeogenesis?
Oxaloacetate (OAA)\
372
What is the main product of the rate limiting step of Fatty Acid Synthesis?
Malonyl-CoA\
373
What is the main therapeutic effect of Bile Acid Resins?
Decrease LDL\
374
What is the major clinical finding of Tay-Sachs disease?
Progressive neurodegeneration\
375
What is the major overall effect of Fibrates on lipid levels?
Decreases TG levels\
376
What is the major overall effect of Fibrates?
Decrease TG\
377
What is the mechanism of action of Ribavirin?
Inhibition of IMP dehydrogenase\
378
What is the most basic amino acid?
Arginine\
379
What is the most common cause of death from Pompe Disease?
Heart failure, specifically hypertrophic cardiomyopathy\
380
What is the most common cause of Homocystinuria?
Cystathionine Synthase deficiency\
381
What is the most common cause of oxidative damage in G6PD deficiency?
Infection\
382
What is the most common initial sign of Gaucher disease?
Hepatosplenomegaly\
383
What is the most common Lysosomal Storage Disease?
Gaucher disease\
384
What is the most common urea cycle disorder?
OTC deficiency\
385
What is the most serious symptom in Maple Syrup Urine Disease?
Neurotoxicity\
386
What is the most severe clinical presentation of Galactokinase Deficiency?
Infantile cataracts\
387
What is the ocular clinical finding in Homocystinuria?
Down and in' lens subluxation\
388
What is the ocular finding in patients with Vitamin B2 (Riboflavin) deficiency?
Corneal vascularization\
389
What is the pathogenesis of Familial Hypercholesterolemia?
Defective LDL receptor; Defective Apo B-100\
390
What is the pathogenesis of Hypertriglyceridemia?
Hepatic overproduction of VLDL\
391
What is the primary metabolic fuel source after 1 minute of maximal effort exericse?
Aerobic metabolism\
392
What is the primary metabolic fuel source in the first 10 seconds of maximal effort exericse?
Creatine phosphate (Phosphocreatinine)\
393
What is the primary metabolic fuel source in the first minute of maximal effort exericse?
Anaerobic metabolism\
394
What is the primary metabolic fuel source in the first second of maximal effort exericse?
Stored ATP\
395
What is the product formed by HMG-CoA Reductase?
Mevalonate\
396
What is the rate limiting enzyme in de novo purine synthesis?
Glutamine-PRPP amidotransferase\
397
What is the rate limiting enzyme in pyrimidine synthesis?
Carbamoyl phosphate synthetase II\
398
What is the rate limiting step for gluconeogenesis?
FBPase-1\
399
What is the shape of Cystine kidney stones?
Hexagonal\
400
What is the source of the carbons in urea?
CO2\
401
What is the storage form of Vitamin D?
25-OH-Vitamin D\
402
What is the treatment for Abetalipoproteinemia?
Restriction of LCFA; Large doses of oral Vitamin E\
403
What is the treatment for cyanide poisoning?
Nitrites + thiosulfate, hydroxocobalamin\
404
What is the treatment for Cystinuria?
Alkalinization of urine; Chelating agents; Hydration\
405
What is the treatment for Hereditary Fructose Intolerance?
Avoid fructose, sucrose, and sorbitol\
406
What is the treatment for Hyperammonemia?
Low protein diet; Lactulose; Antibiotics; Benzoate, phenylacetate, or phenylbutyrate\
407
What is the treatment for Medium Chain Acyl-CoA Dehydrogenase deficiency?
Avoid fasting\
408
What is the treatment for Orotic Aciduria?
Uridine\
409
What is the treatment for Pyruvate Dehydrogenase Complex Deficiency?
Ketogenic diet\
410
What is the treatment for Von Gierke Disease?
Frequent cornstarch ingestion between meals; Avoidance of fructose and galactose\
411
What is the treatment of Hyperchylomicronemia?
Very low fat diet\
412
What is the tremor called in patients with Hyperammonemia?
Asterixis\
413
What is the triad of early symptoms of Fabry disease?
Episodic peripheral neuropathy; Angiokeratomas; Hypohidrosis\
414
What lysosomal enzyme converts glycogen directly into glucose?
Alpha-1,4-glucosidase\
415
What may occur if Gemfibrozil is taken with a HMG-CoA reductase inhibitor?
Rhabdomyolysis\
416
What metabolites are toxic in patients with Phenylketonuria?
Phenylketones\
417
What molecule is a reserve when ATP falls rapidly in muscle cells during exercise?
Phosphocreatine\
418
What molecule is added to Carbamoyl Phosphate to make Orotic acid?
Aspartate\
419
What molecules are the ketone bodies?
Acetoacetate; Acetone; Beta-hydroxybutyrate\
420
What molecules are the origin of the carbon atoms in purine synthesis?
THF; Glycine; CO2\
421
What molecules are the origin of the nitrogen atoms in purine synthesis?
Glycine Aspartate Glutamine; ; ![]()
\
422
What must be present for GKRP to inactivate Glucokinase?
F6P\
423
What poison inhibits lipoic acid?
Arsenic\
424
What stimulates Acetyl-CoA Carboxylase?
Insulin; Citrate\
425
What sympathomimetic in foods can cause a hypertensive crisis in patients on MAOi?
Tyramine\
426
What syndrome has anti-histone antibodies?
Drug-induced Lupus\
427
What tissues in the body do not use fatty acids as a source of energy?
RBCs; Brain\
428
What types of bonds are found between branches of glycogen?
Alpha-(1,6) bonds\
429
What types of bonds are found between linkages of glycogen?
Alpha-(1,4) bonds\
430
What will be seen in a peripheral blood smear in patients with Abetalipoproteinemia?
Acanthocytes\
431
When 3 carbons remain in the fatty acid in Beta-oxidation, what molecule results?
Propionyl-CoA\
432
When an amino group is added to Pyruvate, which structure results?
Alanine\
433
When does screening for Phenylketonuria occur?
2-3 days after birth\
434
When in life do symptoms start occuring in a patient with Classic Galactosemia?
When infant starts breastfeeding\
435
Where are the predominant sites of fatty acid synthesis in the body?
Liver; Lactating mammary glands; Adipose tissue\
436
Where does Ezetimibe block cholesterol absorption?
Intestinal brush border\
437
Where in the body are ketone bodies produced?
Liver\
438
Where in the body does aerobic metabolism of glucose use the glycerol-3-phosphate shuttle?
Muscle\
439
Where in the body does aerobic metabolism of glucose use the malate-aspartate shuttle?
Heart Liver\
440
Where in the body does Na+ dependent entry of glucose occur?
Intestinal epithelium Renal tubules\
441
Where in the body is Glucose-6-Phosphatase found?
Liver; Kidney\
442
Where in the body is glycogen most abundant?
Muscle Liver\
443
Where in the cell do you find alpha-1,4-glucosidase?
Lysosomes\
444
Where in the cell does the HMP Shunt occur?
Cytosol\
445
Where in the nephron is Vitamin D activated?
PCT\
446
Where is cholecalciferol acquired?
Formed in sun-exposed skin; Consumed in milk, fish, plants\
447
Where is ergocalciferol acquired?
Ingested from plants, fungi, yeast\
448
Where is glycogen in excess in Von Gierke Disease?
Liver; Kidney\
449
Where is HDL secreted from?
Liver; Small intestine\
450
Where is Lipoprotein Lipase found?
Vascular endothelial surface\
451
Where is the GLUT-1 transporter found?
Brain; RBC\
452
Where is the GLUT-4 transporter found?
Fat tissue; Skeletal muscle\
453
Where is the hyperpigmentation rash found in patients with a niacin deficiency?
Sun-exposed areas\
454
Where is the location of the hyperpigmentation seen in Hartnup disease?
Sun-exposed regions\
455
Where is the location of the rash seen in Hartnup disease?
C3/C4 dermatomal distribution\
456
Which amino acid is an excitatory neurotransmitter?
Glutamate\
457
Which amino acid is important for heme synthesis?
Glycine\
458
Which amino acid is the recipient of an acetyl group in histone acetylation?
Lysine\
459
Which amino acid is used to synthesize Serotonin?
Tryptophan\
460
Which amino acid metabolic disease can cause a Marfanoid body habitus?
Homocystinuria\
461
Which amino acid metabolism disease can result in a Marfanoid body habitus?
Homocystinuria\
462
Which amino acids are purely ketogenic?
Leucine; Lysine\
463
Which amino acids can enter the TCA cycle via conversion to Propionyl-CoA?
Valine; Methionine; Isoleucine; Threonine\
464
Which anti-seizure drug can cause a Vitamin B9 deficiency?
Phenytoin\
465
Which antibiotics are used to decrease NH3 producing gut bacteria to treat hyperammonemia?
Rifaximin; Neomycin\
466
Which apoproteins on HDL are donated to other lipoproteins?
Apo C-II; Apo E\
467
Which atom(s) does Aspartate contribute in endogenous Purine synthesis?
1 nitrogen\
468
Which atom(s) does CO2 contribute in endogenous Purine synthesis?
1 carbon\
469
Which atom(s) does Glutamine contribute in endogenous Purine synthesis?
2 nitrogens\
470
Which atom(s) does Glycine contribute in endogenous Purine synthesis?
2 carbons, 1 nitrogen\
471
Which atom(s) does THF contribute in endogenous Purine synthesis?
2 carbons\
472
Which auto-antibodies are classically associated with Drug-Induced Lupus?
Anti-histone antibodies\
473
Which autosomal recessive disorder of Zinc malabsorption causes diarrhea and rash?
Acrodermatitis enteropathica\
474
Which B vitamin is stored in the liver for 3-4 months?
Vitamin B9\
475
Which B vitamin is stored in the liver for 3-4 years?
Vitamin B12\
476
Which carbohydrates are taken up into the enterocytes by the SGLT1 transporter?
Glucose; Galactose\
477
Which cell type is characteristically seen in Niemann-Pick disease?
Foam cells\
478
Which cell type is destroyed in Krabbe disease?
Oligodendrocytes\
479
Which cell type synthesizes chylomicrons?
Enterocytes\
480
Which cellular processes are impaired in patients with Von Gierke Disease?
Gluconeogensis; Glycogenolysis\
481
Which class of diuretics can raise lipid levels?
Thiazide diuretics\
482
Which class of lipid drugs upregulates Lipoprotein lipase?
Fibrates\
483
Which conditions have a cherry red spot on the macula on fundoscopy?
Niemann-Pick disease; Tay-Sachs disease; Retinal artery occlusion\
484
Which disease causes Tyrosine to become an essential amino acid?
Phenylketonuria (PKU)\
485
Which disease involves a deficiency in alpha-L-iduronidase?
Hurler syndrome\
486
Which disease involves a deficiency in Iduronate-2-sulfatase?
Hunter syndrome\
487
Which disease involves an absence of an amino acid transporter in the kidney, leading to loss of tryptophan in the urine?
Hartnup disease\
488
Which disease requires avoiding aspartame?
Phenylketonuria (PKU)\
489
Which disorder involves a hereditary deficiency of galactokinase?
Galactokinase deficiency\
490
Which disorder of purine metabolism can manifest with self-mutilating behavior?
Lesch-Nyhan syndrome\
491
Which drug inhibits de novo purine synthesis?
6-Mercaptopurine/ Azathioprine (converted to 6-MP)\
492
Which drug is the most effective drug for increasing serum HDL?
Niacin\
493
Which drug of abuse can trigger a gout attack?
Alcohol\
494
Which drugs are bile acid resins?
Cholestyramine; Colestipol; Colesevelam\
495
Which drugs are PCSK9 inhibitors?
Alirocumab; Evolocumab\
496
Which drugs cause Drug-Induced Lupus?
Methyldopa; Sulfa drugs; Hydralazine; Isoniazid; Procainamide; Phenytoin; Etanercept\
497
Which drugs cause hemolysis in G6PD deficiency?
Sulfonamides; Isoniazid; Dapsone; Primaquine; Aspirin; Ibuprofen; Nitrofurantoin\
498
Which drugs inhibit IMP dehydrogenase?
Ribavirin; Mycophenolate\
499
Which drugs inhibit IMP dehydrogenase?
Ribavirin; Mycophenolate\
500
Which enzyme catalyzes the rate limiting step in Fatty acid oxidation?
Carnitine palmitoyltransferase I (CPT1)\
