BIOCHEMISTRY-Metabolism Flashcards
(561 cards)
1
Q
These are the two sites where the metabolism takes place
A
Mitochondria and Cytoplasm
2
Q
These three process take place in the mitochondria and the cytoplasm
A
Heme synthesis, Urea cycle, Gluconeogenesis
3
Q
Name the processes that take place in the mitochondria
A
Fatty acid oxidation (β oxidation)
Acetyl CoA production, TCA cycle
Oxidative phosphorylation
4
Q
Which processes take place in the cytoplasm?
A
Glycilysis Fatty acid synthesis HMP shunt Protein synthesis Steroid synthesis Cholesterol synthesis
5
Q
What does Glucokinase catalyzes?
A
Catalyzes the phosphorylation of glucose using a molecule of ATP
6
Q
What is the function of Kinase?
A
Uses ATP to add high energy phosphate group onto substrate
7
Q
This enzyme adds inorganic phosphate onto substrate without using ATP
A
Phosphorylase
8
Q
What does the Phosphatase does to the substrate?
A
removes phosphate group
9
Q
This enzyme catalyzes oxidation reduction reactions
A
Dehydrogenase
10
Q
What does Hydroxylase does to the substrate?
A
Adds hydroxyl group (-OH)
11
Q
Which is the effect of Carboxylase?
A
Transfers CO2 groups with help of biotin
12
Q
This enzyme relocates a functional group within a molecule
A
Mutase
13
Q
This enzyme manages the glycolysis
A
Phosphofructokinase-1 (PFK-1)
14
Q
Who are the positive regulators of PFK-1
A
AMP +
Fructose- 2, 6- biphosphate +
15
Q
These are the negative regulators of PFK-1
A
ATP -
citrate -
16
Q
Which process is fructose-1,6 biphosphatase related?
A
Gluconeogenesis
17
Q
ATP and acetyl-CoA which kind of regulators are for fructose-1,6 biphosphatase?
A
positive regulators
18
Q
These are the negative regulators for fructose-1,6 biphosphatase
A
AMP -
Fructose- 2, 6- biphosphate -
19
Q
Which are the alternative names for Citric acid cycle?
A
Tricarboxylic acid cycle (TCA) or Krebs cycle
20
Q
This enzyme has a great importance on TCA cycle
A
Isocitrate dehydrogenase
21
Q
This is the positive regulator in Citric acid cycle
A
APD+
22
Q
Who are the negative regulators in Krebs cycle?
A
ATP- and NADH
23
Q
This enzyme manages the glycogenesis
A
Glycogen synthase
24
Q
These are positive regulators in glycogenesis
A
Glucose-6-phosphate
Insulin
Cortisol
25
Epinephrine and glucagon are negative regulators in this process
Glycogenesis
26
Which process is managed by glycogen phosphorylase?
Glycogenolysis
27
Glucose-6-phosphate, Insulin and ATP are negative regulators for this process
Glycogenolysis
28
Who are positive regulators in Glycogenolysis?
AMP, Epinephrine and glucagon
29
This enzyme is related to hexose monophosphate shunt...
Glucose-6-phosphate dehydrogenase (G6PD)
30
Is NADP a positive or negative regulator in pentose phosphate pathway?
Positive
31
It´s an alternative name for hexose monophosphate shunt...
Pentose phosphate pathway
32
Is NADPH a positive or negative regulator in hexose monophosphate shunt?
Negative
33
Which process is Carbamoyl phosphate synthetase II related to?
De novo pyrimidine synthesis
34
This enzyme is related to De novo purine synthesis
Glutamine-phosphoribosylpyrophosphate (PRPP) amidotransferase
35
These are negative regalutors in De Novo purine synthesis
AMP, IMP, GMP
36
The carbamoyl phosphate synthetase I is related to this process
Urea Cycle
37
The N-acetylglutamate is a positive regulator in this process
Urea cycle
38
Which enzyme is related to fatty acid oxidation?
Carnitine acyltransferase I
39
Who is a negative regulator in Fatty acid synthesis?
Glucagon and palmitoyl-CoA
40
Act as an enzyme in the Fatty acid synthesis
Acetyl CoA carboxylase (ACC)
41
Insulin and citrate are postive regulators in fatty acid oxidation or fatty acid synthesis
In the fatty acid synthesis
42
Which process has HMG- CoA synthase as an enzyme?
Ketogenesis
43
Which enzyme acts in Cholesterol synthesis?
HMG- CoA reductase
44
Insulin and Thyroxine are positive regulators in this process
Cholestherol synthesis
45
Which are the negative regulators in Cholestherol synthesis?
Glucagon, cholesterol
46
How many ATP are produced in the aerobic metabolism via malate aspartate shuttle?
32 net ATP
47
Which organs are related to aerobic metabolism via malate aspartate shuttle?
Heart and liver
48
How many ATP are produced in the aerobic metabolism via glycerol 3 phosphate?
30 net ATP
49
Which tissue is related to aerobic metabolism via glycerol 3 phosphate?
Muscle
50
How many ATP are produced in the anaerobic glycolysis?
2 net ATP per glucose molecule
51
Can ATP hydrolysis be coupled to energetically favorable reactions?
False, ATP hydrolysis can be coupled to energetically unfavorable reactions
52
Which element causes glycolysis to produce zero net ATP?
Arsenic
53
From which pathway is NADPH produce?
HMP shunt
54
Where is used NADPH?
Anabolic processes
Respiratory burst
Cytochrome P450 system
Glutathione reductase
55
In which processes is NAD+ generally used?
Catabolic processes
56
During the catabolic processes which is the function for NAD+ ?
To carry reducing equivalents away as NADH
57
In which processes is NADPH generally used?
Anabolic processes
58
These are examples of anabolic processes
Steroid and fatty acid synthesis
59
During the anabolic processes which is the function for NADPH?
As a supply of reducing equivalents
60
Which is the first step of glycolysis?
Phophorylation of glucose to yield glucose 6-P
61
In which other process Phophorylation of glucose to yield glucose 6-P is the first step?
Glycogen synthesis in the liver
62
Which enzyme catalyses Phophorylation of glucose to yield glucose 6-P?
Hexokinase or glucokinase depending in the tissue
63
At low glucose concetrations who sequesters glucose in the tissue?
Hexokinase
64
When there is high glucose concentration, who stores glucose excess?
The liver
65
These are the location of action of the Glucokinase
Liver, β cells of pancreas
66
Which are the location of action of the Hexokinase?
Most tissues, but not Liver nor β cells of pancreas
67
What is the difference in Km between Hexokinase and Glucokinase
Hexokinase has lower Km but ↑ affinity
| and Glucokinase has Higher Km but ↓ affinity
68
For the Glucokinase what does it meas that has Higher Km but ↓ affinity?
This means that it operates only when serum glucose levels are high.
69
If tissues need to use glucose at lower serum levels which enzyme is used?
Use the higher affinity (lower Km) hexokinase
70
How is the Vmax in the Hexokinase
Lower and decrease Capacity
71
Higher V max with increase capacity.... Glucokinase or Hexokinase?
Glucokinase
72
Feedback inhibited by glucose 6 P....Glucokinase or Hexokinase?
Hexokinase
73
Gene mutation associated with maturity onset diabetes of the young... Glucokinase or Hexokinase?
Glucokinase
74
Which is the equation in the net glycolysis?
Glucose+ 2Pi+ 2ATP+ 2 NAD→ 2 pyruvate+ 2 ATP+ 2 NADH+ 2 H+ 2 H2O
75
Is the equation in the net glycolysis balanced?
Not balanced chemically
76
On what depends the exact balance equation in the net glycolysis?
Ionization state of reactants and products
77
For the glucose conversion to glucose 6 phosphate is needed the hexokinase/glucokinase, what else is required?
ATP
78
For the fructose 6 P conversion to fructose 1,6 BP is required ATP, wich enzyme catalyzes this reaction?
Phosphofructokinase
79
Which processes produces ATP during the glycolysis?
1,3 BPG→ 3PG
| Phosphoenolpyruvate → Pyruvate
80
Which enzyme catalyzes the conversion from Phosphoenolpyruvate → Pyruvate?
Pyruvate kinase
81
Which enzymes manages the conversion from fructose 2, 6 biphosphate to Fructose 6 phosphate?
Fructose biphosphatase 2 (FBPase2)
82
When does Fructose biphosphatase 2 (FBPase2) is activated?
Activated in fasting state
83
Which enzymes manages the conversion from Fructose 6 phosphate to fructose 2, 6 biphosphate?
Phosphofructokinase-2 (PFK 2)
84
When does Phosphofructokinase-2 is activated?
Activated during fed state
85
This enzymes are the same bifunctional enzyme whose function is reversed by phosphorylation by protein kinase A
Phosphofructokinase-2 (PFK 2) and Fructose biphosphatase 2 (FBPase2)
86
During the Fasting state which process is increase, Glycolysis or Gluconeogenesis
Gluconeogenesis
87
Who is primary increase during the fasting state?
Increase Glucagon
88
The increase of Glucagon during fasting state increases cAMP, after this... who is increased?
↑ protein kinase A
89
If the protein Kinase A is increased during fasting, which enzyme is increased and which one decreased?
Fructose biphosphatase 2 (FBPase2) is increased
| Phosphofructokinase-2 (PFK 2) is decreased
90
Who is primary increase during the fed state?
Increase Insulin
91
The increase of insulin during fed state decreases cAMP, after this... who is decreased?
↓ protein kinase A
92
If the protein Kinase A is decreased during feeding, which enzyme is increased and which one decreased?
Fructose biphosphatase 2 (FBPase2) is decreased
| Phosphofructokinase-2 (PFK 2) is increased
93
This mitochondrial enzyme complex links the glycolysis and TCA cycle?
Pyruvate dehydrogenase complex
94
When does the Pyruvate dehydrogenase complex is activated?
Active in fed state
95
Which is the reaction catalyzed by Pyruvate dehydrogenase complex?
pyruvate+ NAD+ CoA→ acetyl-CoA+CO2 + NADH
96
In the mitochondrial enzyme complex which links the glycolysis and TCA cycle, how many enzymes and cofactors it contains?
Contains 3 enzymes that require 5 cofactors
97
Which are the 5 cofactors in the Pyruvate dehydrogenase complex?
```
Pyrophosphate (B1 thiamine; TPP)
FAD (B2 riboflavin)
NAD (B3 NIacin)
CoA (B5 pantothenate)
Lipoic Acid
```
98
What happens to NAD/ NADH ratio, ADP and Ca2+ during exercise in the Pyruvate dehydrogenase complex?
They´re increased
99
The Pyruvate dehydrogenase complex to which complex is it similar?
α ketoglutarate dehydrogenase complex
100
In the TCA cycle what is the final product of α ketoglutarate
Succinyl CoA
101
Which elemet inhibits lipoic acid?
Arsenic
102
During the inhibition caused by Arsenic to lipoic acid which symptoms are common?
Vomiting, rice watter stools, garlic breath
103
If there is a Pyruvate dehydrogenase complex deficiency, which would be the consequences?
Causes a buildup of pyruvate that gets shunted to lactate (via LDH) and alanine (via ALT)
104
Which are the only purely ketogenic aminoacids?
Lysine and Leucine
105
Which findings are common during the Pyruvate dehydrogenase complex deficiency?
Neurologic defects, lactic acidosis, increase alanine starting in infancy
106
This is the treatment for Pyruvate dehydrogenase complex deficiency
Increase intake of ketogenic nutrients (high content or increase lycine and leucine)
107
From Glucose we can get pyruvate, but from pyruvate what do we get?
Alanine
Oxaloacetate
Acetyl CoA
Lactate
108
Which pyruvate product is related to Cahill cycle?
Alanine
109
This pyruvate product is the result of Cori cycle
Lactate
110
Which enzyme and cofactor helps during the pyruvate metabolic pathway in order to get Alanine?
Alanine aminotransferase (B6 as a cofactor)
111
What is the importance of alanine?
It carries amino groups to the liver from muscle
112
Which enzyme and cofactor helps during the pyruvate metabolic pathway in order to get Oxaloacetate?
Pyruvate carboxylase (biotin as cofactor)
113
What is the importance of Oxaloacetate?
Can replenish TCA cycle or be used in gluconeogenesis
114
Which enzyme and cofactor helps during the pyruvate metabolic pathway in order to get Acetyl CoA?
Pyruvate dehydrogenase (B1, B1, B3, B5, lipioc acid)
115
What is the importance of Pyruvate dehydrogenase?
Transition from glycolysis to the TCA cycle
116
Which enzyme and cofactor helps during the pyruvate metabolic pathway in order to get Lactate?
Lactic acid dehydrogenase (B3)
117
End of anaerobic glycolysis
Lactate
118
This is the step preview to entering the Kreb cycle
Pyruvate→ Acetyl Co'A
119
During the transition from pyruvate → Acetyl CoA in the TCA cycle, what is produce?
1 NADH and 1 CO2
120
Where does the Krebs cycle takes place?
Mitochondria
121
Which are the results from the TCA cycle?
3 NADH, 1 FADH2, 2 CO2, 1 GTP per acetyl CoA
122
How much ATP is needed for each acetyl CoA?
10
123
Who requires the same cofactors as the pyruvate dehydrogenase complex?
α ketoglutarate
124
Which cofactors does α ketoglutarate needs?
B1, B2, B3, B5, lipoic acid
125
Which is the mnemonic for the Krebs cycle?
Citrate Is Krebs Starting Substrate For Making Oxaloacetate
126
Which enzyme is required for Acetyl CoA to get to Citrate?
Citrate synthase
127
Which enzyme is required for Oxalatoacetate to get to Citrate?
Citrate synthase
128
Which enzymes are irreversible during Krebs cycle?
Citrate synthase
Isocitrate dehydrogenase
α KG dehydrogenase
129
Which are the substrates from TCA cycle?
```
Citrate
Isocitrate
α Ketoglutarate
Succinyl CoA
Succinate
Fumarate
Malate
Oxaloacetate
```
130
The NADH electrons produce from glycolysis, where do they go?
Thet enter the mitochondria
131
How does the NADH electrons produce from glycolysis enter the mitochondria?
Via the malate aspartate or glycerol 3 phosphate shuttle
132
Where are transfered the FADH2 electrons in the mitochondria?
To complex II
133
In the mitochondria, who is transfered at lower energy the FADH2 or NADH electrons?
FADH2
134
What is the result of the passage of electrons in the mitochondria?
Results in the formation of proton gradient
135
What is the result of proton gradient coupled to oxidative phosphorylation?
Production of ATP
136
On what consists the complex II?
Succinate dehydrogenase
137
Who inhibits the complex IV in the mitochondria?
Cyanide
| CO
138
Antimycin A inhibits this complex in the mitochondria
Complex III
139
Rotenone inhibits Mitochondria complex V
False Rotenone inhibits Compex I
140
Who inhibits Mitochondria Complex V?
Oligomycin
141
What is produce by Mitochondria complex I, III, IV and V?
H+
142
Acts as a proton ionophore, an agent that can shuttle protons (hydrogen cations) across biological membranes. It defeats the proton gradient across mitochondria and chloroplast membranes.
2,4 Dinitrophenol
143
By which enzyme is ATP produce?
ATP synthase
144
How many ATP are produce for each NADH?
2.5 ATP
145
By each FADH2 how many ATPs are produce?
1.5 ATP
146
This poisoning directly inhibit electron transport, causing a ↓ proton gradient and block of ATP synthesis
Rotenone
Cyanide
Antymicin A
CO
147
Directly inhibit mitochondrial ATP synthase, causing an ↑ proton gradient
Oligomycin
148
Why it isn´t produce any ATP by Olygomycin?
Electron transport stops
149
Produces heat. Increase permeability of memebrane, causing a decrease proton gradient and increase O2 consumption
2, 4 Dinitriphenol
Aspirin
Thermogenin
150
With the 2, 4 Dinitriphenol how is the ATP synthesis and the electron transport?
ATP synthesis stops
| Electron transport continues
151
Which is the illicitly use for 2, 4 Dinitriphenol?
For weight loss
152
Which is a common secondary effect of aspirin after an overdose?
Fever
153
Where do we find thermogenin?
In brown fat
154
Which are the irrevesible enzymes during the gluconeogenesis?
Pyruvate Carboxylase
Phosphoenolpyruvate carboxykinase
Fructose 1,6 bisphosphatase
Glucose 6 phosphate
155
In the gluconeogenesis, where does the pyruvate→ oxaloacetate is performed?
In mitochondria
156
In the gluconeogenesis, which enzymes catalyzes pyruvate→ oxaloacetate?
Pyruvate carboxylase
157
In the gluconeogenesis, which cofactors does pyruvate carboxylase needs to catalyze pyruvate→ oxaloacetate?
Biotin
| ATP
158
Who activates Pyruvate carboxylase?
Acetyl CoA
159
In the gluconeogenesis, where does the oxaloacetate → Phosphoenolpyruvate is performed?
In Cytosol
160
In the gluconeogenesis, which enzymes catalyzes oxaloacetate → Phosphoenolpyruvate?
Phosphoenolpyruvate carboxykinase
161
In the gluconeogenesis, what does Phosphoenolpyruvate carboxykinase needs to catalyze oxaloacetate → Phosphoenolpyruvate
Requires GTP
162
In the gluconeogenesis, where does the Fructose 1,6 BP→ Fructose 6 phosphate is performed?
In cytosol
163
In the gluconeogenesis, which enzymes catalyzes Fructose 1,6 BP→ Fructose 6 phosphate?
Fructose 1-6 biphosphatase
164
In the gluconeogenesis, which cofactors positively stimulates Fructose 1-6 biphosphatase to catalyze Fructose 1,6 BP→ Fructose 6 phosphate
Citrate
165
In the gluconeogenesis, which cofactors inhibits Fructose 1-6 biphosphatase to catalyze Fructose 1,6 BP→ Fructose 6 phosphate
Fructose 2,6 biphosphate
166
In the gluconeogenesis, where does the Glucose 6 P→ Glucose?
In Endoplasmic Reticulum
167
In the gluconeogenesis, which enzymes catalyzes Glucose 6 P→ Glucose?
Glucose 6 phosphatase
168
Primarily where does the gluconeogenesis happens? What is it purpose?
Occurs primarily in the liver, serves to mantain euglycemia during fasting.
169
Which other organs can we find the irrevesible enzymes of the Gluconeogenesis?
Kidney, intestinal ephithelium.
170
What happens if there is a deficiency of the key glucogenic enzymes?
Hypoglicemia
171
Why can´t the muscle participate in gluconeogenesis?
Because it lacks glucose 6 phosphatase
172
Which other route serves as glucose source?
Odd chain fatty acids yield 1 propionyl CoA during metabolism, which can enter the TCA cycle (as succinyl CoA) undergo gluconeogenesis
173
Why can´t the Even chain fatty acids produce new glucose?
Since they yield only acetyl CoA equivalents
174
Provides a source if NADPH from abundantly avaible glucose 6-P
HMP shunt (pentose phosphate pathway)
175
What is required for the reductive reactions?
NADPH
176
This are examples of reductive reactions that need NADH
Glutathione reduction inside RBCs, fatty acid and cholesterol biosynthesis
177
What else does the HMP shunt yields?
Yields ribose for nucleotide synthesis and glycolytic intermediates
178
Which are the distinctic phases in the pentose phosphate pathway
Oxidative and nonoxidative
179
Where do the oxidative and nonoxidative phases of the HMP shunt occurs?
cytoplasm
180
How many ATPs are produced and used during the HMP shunt?
No ATP is used or produced
181
These are the sites where the pentose phosphate pathway take place
Lacting mamary glands, liver, adrenal cortex (sites of fatty acid or steroid synthesis) RBCs
182
In the oxidative and nonoxidative reactions in the HMP shunt, Which one is irreversible?
Oxidative
183
In the oxidative reaction in the pentose phosphate pathway, this is the start point
Glucose 6 Phosphate
184
These two metabolic pathways have Glucose 6 phosphate as the start point
Glycolysis and pentose phosphate pathway
185
Which are the products from the oxidative phase in the HMG shunt?
CO2
2 NADPH
Ribulose 5 P
186
Glucose 6 P dehydrogenase is one of the main enzymes in which metabolic process?
pentose phosphate pathway
187
Who stimulates and who inhibits the oxidative phase in the HMG shunt?
NAPD stimulates
| NADPH exces inhibits
188
This is the end point of the oxidative reaction, but also the start point in the nonoxidative reaction in the pentose phosphate pathway
Ribulose 5 P
189
Which are the final products of the nonoxidative phase with the Ribulose 5 P?
Ribose 5 P
G3P
F6P
190
Which enzyme is needed for Ribulose 5 P to get to the final products?
Phosphopentose isomerase transketolases
191
Which cofactor is required for Ribulose 5 P to get to the final products?
Requires B1
192
In the Respiratory burst what does it involves?
Involves the activation of the phagocyte NADPH oxidase complex
193
What other name does the respiratory burst receives?
Oxidative burst
194
During the Respiratory burst, which is the substrate for the phagocyte NADPH oxidase complex?
O2
195
These cells are example of phagocyte NADPH oxidase complex
Neutrophils, monocytes
196
What is the importance of the respiratory burst?
Plays an important role in the immune response→ rapid release of reactive oxygen species
197
Who plays an important role in the creation and neutralization of Reactive Oxigen Species (ROS)?
NADPH
198
Who gives the blue green heme containing pigment to the sputum?
Myeloperoxidase
199
Which disease has NADPH oxidase deficiency?
Chronic granulomatous disease (CGD)
200
What happens to the phagocytes of patients with Chronic Granulomatous disease (CGD)?
Phagocytes can utilizr H2O2generated by invading organism and convert it to ROS
201
The CGD patients are at higher risk for infection, from whom ?
Catalase + species
202
Which benefit Catalase + species have against phagocytes?
Catalase + species are capable of neutralizing their own H2O2, leaving phagocytes without ROS for fighting infections
203
Which bacterias are examples of catalase + species?
S. Aureus
| Aspergillus
204
Can P. aeruginosa kill competing microbes? How?
Pyocyanine of P. aeruginosa functions to generate ROS to kill competing microbes
205
This protein inhibits microbial growth via iron chelation
Lactoferrin
206
Where do we find Lactoferrin?
In secretory fluids and neutrophils
207
What is the relationship between NADPH and glutathione?
NADPH is necessary to keep glutathione reduced
208
What repercussion has that NADPH reduces glutathione?
Detoxifies free radicals and peroxides
209
What happens to the RBC if there is a decrease in NADPH?
Leads to an hemolytic anemia due to poor RBC defence agianst oxidizing agents
210
Give examples of oxidizing agents that can lead to hemolytic anemia?
Fava beans, sulfonamides, primaquine, antituberculosis drugs
211
What else can precipitate hemolysis and it´s worst if there is a decrease of NADPH?
Infection
212
How does infection can lead to hemolysis?
Free radicals generated via inflamatory response can diffuse into RBCs and cause oxidative damage
213
If there is a Glucose 6 phosphate dehydrogenase deficiency, which would be the results?
A lack of NADPH, and with that a glutathione increased resulting in increased radicals and peroxides
214
Which kind of genetic disorder is Glucose 6 phosphate dehydrogenase deficiency?
X linked recessive disorder
215
Which is the most common human enzyme deficiency?
Glucose 6 phosphate dehydrogenase deficiency
216
In whom is the glucose 6 phosphate dehydrogenase deficiency more prevalent?
Among blacks
217
How is related the glucose 6 phosphate dehydrogenase deficiency to the malaria?
Increases malarial resistance
218
What is the product of the Glucose 6 phosphate after 6 phosphate dehydrogenase catalazes it?
6 phosphogluconate and NADH
219
Oxidized Hemoglobin precipitaed within RBCs
Heinz bodies
220
Result from the phagocytic removal of Heinz bodies by splenic macrophages
Bite cells
221
Are consider Disorders of fructose metabolism
Essential fructosuria
| Fructose intolerance
222
Involves a defect in fructokinase
Essential fructosuria
223
The essential fructosuria, which kind of mode of inheritance does it follows?
Autosomal recessive
224
Clinically, how is consider the Essential fructosuria?
A benign, asymptomatic condition
225
Why is the essential fructosuria a benign asymptomatic condition?
Because fructose isn´t trapped in cells
226
Which would be the findings in Essential fructosuria?
Fructose in blood and urine
227
Which disorder of the metabolism causes less severe symptoms... Fructose or Galactose disoder?
Disorders of the fructose metabolism cause milder symptoms than analogous disorder of galactose metabolism
228
Which is the cause of hereditary fructose intolerance?
Hereditary deficiency of aldolase B
229
The fructose intolerance, which kind of mode of inheritance does it follows?
Autosomal recesive
230
Where is carried the fructose metabolism?
Liver
231
If there is a lack of aldolase B, who is accumulated?
Fructose 1 P
232
What happens if there is a Fructose 1 P accumulation?
Decrease in avaible phosphate
233
Which cycles are inhibited if there is a lack in phosphate due to Fructose 1 P accumulation?
Glycogenolysis
| Gluconeogenesis
234
When do the symptoms present in the fructose intolerance?
Following consumption of fruit, juice or honey
235
How do we expect the urine dipstick be in Fructose intolerance?
Negative (test for glucose urine only)
236
Which are the symptoms found in Fructose intolerance
Hypoglicemia
Jaundice
Cirhosis
Vomiting
237
For fructose intolerance the treatment is just decrease the intake of fructose
Decrease the intake of fructose and sucrose
238
Which are the compounds of sucrose?
Glucose + fructose
239
Which are the disorders of galactose metabolism?
Galactokinase deficiency
| Classic galactosemia
240
In order to get Galactose 1 P from Galactose which enzyme is needed?
Galactokinase
241
If we have a Galactokinase deficiency and still continue having galactose in our diet, Who is going to accumulate?
Galactitol
242
Which mode of inheritance does Galactokinase deficicency follows?
Autosomal Recessice
243
Which findings can be expected from Galactokinase deficicency?
Galactose appears in blood and urine, infantile cataracts
244
Initially how are manifested the infantile cataracts in the Galactokinase deficicency?
Failure to track objects or to develop a social smile
245
Which is the cause of the classic galactosemia?
Absence of Galactose 1 phosphate uridyltransferase
246
Which mode of inheritance does classic galactosemia follows?
Autosomal recessive
247
How is the damaged caused in classic galactosemia?
By Accumulation of toxic substances (including galactitol, which accumulates in the lens of the eye)
248
Failure to thrive, jaundice hepatomegaly, infantile caracts, intellectual disability are symptoms of which kind of metabolism disorder
Classic galactosemia
249
What kind of diet does the Classic galactosemia patient needs to follow?
Exclude galactose and lactose
250
Which are the compounds of lactose?
(galactose + glucose)
251
Where does the most serious defect can leads in classic galactosemia?
It can lead to PO4 depletion
252
The classic galactosemia which kind of infection can lead in neonates?
E. Coli Sepsis in neonates
253
An alternative method of trapping glucose in the cell is to convert it to its alcohol counterpart, How is named this alcohol counterpart?
Sorbitol
254
Which is the enzyme that catalizes the convertion from glucose to sorbitol?
Aldose reductase
255
Which is the substrate form by sorbitol after Sorbitol dehydrogenase catalyzes it?
Fructose
256
Which tissues have aldose reductase and sorbitol dehydrogense?
Liver, ovaries, and seminal vesicles have both enzymes
257
What happens if there is an insufficient amount of aldose reductase and sorbitol dehydrogense in some tissues?
Sorbitol accumulates, causing osmotic damage
258
Which symptomps are seen with sorbitol accumulation?
Cataracts, retinopàthy and peripheral neuropathy seen with chronic hyperglycemia in diabetes
259
Aldose reductase in which metabolic pathways is related?
Glucose → Sorbitol
| Galactose → Galacitol
260
Which tissues just have Aldose reductase?
Schwann cells, retinam and kidneys
261
Where does the lactase functions to digest lactose?
On the brush border
262
Lactase digests lactose in which compounds?
Glucose and galactose
263
In what consists primary lactase deficiency?
Age dependent decline after childhood (absence of lactase persistent allele)
264
In which kind of people is common the lactase deficiency?
Asian, African or Native American descent
265
What happens in the secondary lactase deficiency?
Loss of brush border due to gastroenteritis (rotavirus), autoimmune disease
266
Which are the causes of Lactase deficiency?
Primary
Secondary
Congenital
267
What does the lactase deficency demonstrate in the stools?
↓ pH
268
What does the lactase deficency shows breath?
↑ Hydrogen content with lactose tolerance test
269
What does intestinal biopsy reveals in Lactase intolerance?
Normal mucosa in patients with lactose intolerance
270
Bloating, cramps, flatulence, osmotic diarrhea are the main symptomps of which metabolic disorder?
Lactase deficiency
271
Which is the management of Lactase deficiency?
Avoid dairy products or add lactase pills to diet; lactose free milk
272
Which kind of aminoacids are found in proteins?
Only L form amino acids
273
Which is the classification of essential aminoacids?
Glucogenic, Glucogenic/ketogenic, and ketogenic
274
These essential aminoacid are Glucogenic
Methionine, Valine, Histidine
275
These essential aminoacids are consider glucogenic/ ketogenic aminoacids
Isoleucine, phenylalanine, threonine, thypthophan
276
Which are examples of Ketogenic aminoacids?
Leucine, Lysine
277
Which are the three types of Amino acids?
Essential
Acidic
Basic
278
Give examples of acidic amino acids
Aspartic acid and glutamic acid
279
Which is the characteristic of the acidic aminoacids?
Nagatively charged at body pH
280
These are examples of basic amino acids
Arginine, lysine and histidine
281
Which of the three basic amino acids is the most basic?
Arginine
282
Which of the three basic amino acids has no charge at body pH?
Histidine
283
Which basic amino acids are required during periods of growth?
Arginine and histidine
284
Which basic amino acids are increased in histones? Why are they important?
Arginine and Lysine, which bind negatively charged DNA
285
Which amino acids need to be supplied in diet?
All essential amino acids
286
Why is it important the amino acid catabolism?
Results in the formation of common metabolites (pyruvate, acetyl CoA) which serve as metabolic fuels
287
What happens to the excess nitrogen (NH3) generated by Amino acid catabolism?
Is converted to urea and excreted by the kidneys
288
What is the name of the process where the excess nitrogen (NH3) is converted to urea and excreted by the kidneys?
Urea cycle
289
During the Urea cycle from the Starting point NH3 + CO2, which is the next step? and which enzyme is used? which is the cofactor required?
Carbamoyl phosphate is the substrate
Carbamoyl phosphate synthetase I is the enzyme
N acetylglutamate the cofactor
290
Where are realized the first step of the Urea cycle?
Mitochondria
291
Which substrate is formed thanks to Carbamoyl phosphate and Ornithine by Ornithine transcarbamylase?
Citruline
292
Which are the substrate of Urea cycle?
```
Ornithine
Carbamoyl phosphate
Citruline
Asoartate
Argininosuccinate
Fumarate
Arginine
Urea
```
293
Who transports amonia?
Alanine and glutamate
294
Is the Hyperammonemia acquired or hereditary?
Both
295
How does Hyperammonemia can be aquired?
By liver disease
296
This is an example of how you get hereditary hyperammonemia
Urea cycle deficiencies
297
Results in excess NH4 which depletes α ketoglutarate
Hyperammonemia
298
Which cycle is inhibited by Hyperammonemia ?
The Excess of NH4 which depletes α ketoglutarate leads to inhibition of TCA cycle
299
Which diet is recomended in hypermmonemia?
Limit protein in diet
300
Which medicines may decrease amonia levels?
Benzoate and phenylbutyrate
301
How does Benzoate and phenylbutyrate decrease amonia levels?
Both bind amino acid and lead to excretion
302
What helps to acidify the GI tract and trap NH4 for excretion?
Lactulose
303
Which symptomps are seen in Amonia intoxication?
Tremor (asterixis), slurring of speech, somnolenc, vomiting, cerebral edema, blurring vision
304
Required cofactor for carbamoyl phosphate synthetase I?
N acetylglutamate
305
The absence of N acetylglutamate in which disease can it end?
Hyperammonemia
306
N acetylglutamate deficiency presention is identical to which other deficiency?
Carbamoyl phosphate synthetase I deficiency
307
How can you differentiate N acetylglutamate deficiency and Carbamoyl phosphate synthetase I deficiency?
↑ ornithine with normal urea cycle enzymes suggest hereditary N acetyl glutamate deficiency
308
Which is the most common urea cycle disorder?
Ornithine transcabamylase deficiency
309
Which mode of inheritance does Ornithine transcabamylase deficiency follows?
X linked recessive
310
With what does the Ornithine transcabamylase deficiency interferes in the body?
Interferes with body´s ability to eliminate ammonia
311
Who transports amonia?
Alanine and glutamate
312
Is the Hyperammonemia acquired or hereditary?
Both
313
How does Hyperammonemia can be aquired?
By liver disease
314
This is an example of how you get hereditary hyperammonemia
Urea cycle deficiencies
315
Results in excess NH4 which depletes α ketoglutarate
Hyperammonemia
316
Which cycle is inhibited by Hyperammonemia ?
The Excess of NH4 which depletes α ketoglutarate leads to inhibition of TCA cycle
317
Which diet is recomended in hypermmonemia?
Limit protein in diet
318
Which medicines may decrease amonia levels?
Benzoate and phenylbutyrate
319
How does Benzoate and phenylbutyrate decrease amonia levels?
Both bind amino acid and lead to excretion
320
What helps to acidify the GI tract and trap NH4 for excretion?
Lactulose
321
Which symptomps are seen in Amonia intoxication?
Tremor (asterixis), slurring of speech, somnolenc, vomiting, cerebral edema, blurring vision
322
Required cofactor for carbamoyl phosphate synthetase I?
N acetylglutamate
323
The absence of N acetylglutamate in which disease can it end?
Hyperammonemia
324
N acetylglutamate deficiency presention is identical to which other deficiency?
Carbamoyl phosphate synthetase I deficiency
325
How can you differentiate N acetylglutamate deficiency and Carbamoyl phosphate synthetase I deficiency?
↑ ornithine with normal urea cycle enzymes suggest hereditary N acetyl glutamate deficiency
326
Which is the most common urea cycle disorder?
Ornithine transcabamylase deficiency
327
Which mode of inheritance does Ornithine transcabamylase deficiency follows?
X linked recessive
328
With what does the Ornithine transcabamylase deficiency interferes in the body?
Interferes with body´s ability to eliminate ammonia
329
When is evident the ornithine transcarbamylase deficiency?
Often evident in the first few days of life, but may present with late onset
330
If there is an excess of carbamoyl phosphate in what is it converted?
Orotic Acid
331
Which pathway is orotic acid related?
Part of the pyrimidine synthesis pathway
332
↑ Orotic Acid in blood and urine, ↓ BUN, symptoms of hyperammonemia
Which are the findings in ornithine transcabamylase deficiency?
333
This finding helps to diferentiate ornithine transcabamylase deficiency and Orotic Aciduria
No megaloblastic anemia in ornithine transcabamylase deficiency
334
Which is the chain of derivatives from the amino acid Phenylalanine?
Phenylalanine→ Tyrosine → Dopa → Dopamine→ NE→ Epi
335
Which other derivative does Tyrosine has, apart from Dopa?
Thyroxine
336
What other derivative does Dopa has, other than Dopamine?
Melanin
337
The amino acid tryptophan has two derivatives, who are they?
Niacin
| Serotonin
338
Which products can Niancin could have?
NAD+/ NADP+
339
Melatonin is the derivative of whom?
Serotonin
340
Which amino acid is the precursos of Histamine?
Histidine
341
This aminoacid is important in the Heme synthesis
Glycine
342
What is the result of Glycine after B6 works as a cofactor
Porphyrin
343
Glutamate can have two dericatives, who are they?
GABA
| Glutathione
344
This three are the derivatives if Arginine
Creatinine
Ura
Nitric Oxide
345
Which enzyme catalyzes the reaction from Phenylalanine to tyrosine
Phenylalanine hydroxylase
346
If there is a deficiency in Phenylalaline hydroxylase which disease can be caused?
Phenylketonuria
347
Who is the precursor of Homogentisic acid?
Tyrosine
348
After Homogentisate oxidase catalyzes Homogentisic acid, Who is the result?
Maleylacetoacetic acid
349
Which disease is caused if there is a deficiency in Homogentisate oxidase?
Alkaptonuria
350
Which cycle is Maleylacetoacetic acid precursor?
TCA cycle
351
From Tyrosine to DOPA which enzyme acts?
Tyrosine hydroxylase
352
What is the meaning of DOPA?
Dihydroxyphenylalanine
353
If Melanin is a derivate of DOPE, which enzyme is affected in albinism in this conversion?
Tyrosinase
354
Which enzyme acts in the catalization from DOPA to Dopamine?
Dopa decarboxylase
355
Which medicine inhibits Dopa decarboxylase in the conversion of dopamie from DOPA?
Carbidopa
356
Which cofactor is needed to get Norepinephrine from Dopamine
Vitamin C
357
In the catecholamine synthesis, S adenosyl Methionine is needed to obtain...
Norepinephrine → Epinephrine
358
This suprarenal cortex product stimulates positively the conversion from Norepinephrine to Epinephrine
Cortisol
359
Methanephrine is the direct result of Norepinephrine or Epinephrine?
Epinephrine results in Metanephrine
| Norepiephrine results in Normetanephrine
360
Which is the common result of Metanephrine
| and Normetanephrine?
Vanillylmandelic acid
361
Homovanilic acid comes from?
Dopamine
362
Which are the causes of Phenylketonuria?
Due to ↓ phenylalanine hydroxylase or ↓ tetrahydrobiopterin cofactor
363
Which is consider the malignant PKU?
↓ tetrahydrobiopterin cofactor
364
What is increase in the urine thanks to the increase of phenylalanine?
Phenylketones in urine
365
Which are the findings in PKU?
Intellectual disability, growth retardation , seizures, fair skin, eczema, musty body odor
366
What is the treatment in PKU patients?
↓ Phenylalanine and ↑ Tyrosine in diet
367
What kind of inheritance pattern does PKU follows?
Autosomal recessive
368
Which would be the incidence of PKU?
1: 10, 000
369
After birth when does the neonate has to be screen in PKU?
Screened 2-3 days after birth
370
Why the PKU neonates are normal at birth?
Because of maternal enzyme during fetal life
371
Which kind of artificial sweetner does the PKU patients must avoid?
Artificial sweetner aspartame, which contains phenylalanine
372
This is the cause of maternal PKU?
Lack of proper dietary therapy during pregnancy
373
Microcephaly, intellectual disability, growth retardation, congenital heart defects are findings of?
Maternal PKU
374
Give the explanation of Alkaptonuria
Congenital deficiency of homogentisate oxidase in the degradative pathway of tyrosine to fumarate
375
This is mode of inheritance in Alkaptonuria
Autosomal recessive
376
Which other names doers Alkaptonuria receives?
Ochronosis
377
The alkaptonuria, is it a benign or malignant disease?
Benign disease
378
How is made the cystine?
Made of 2 cysteines connected by a disulfide bond
379
The homocystinuria follows this mode of inheritance
Autosomal recessive
380
Name the three types of homocystinuria
```
Cystathionine synthase deficiency
↓ Affinity of cystathionine synthase for pyridoxal phosphate
Homocysteine methyltransferase (methionine synthase) deficiency
```
381
The treatment of this homocysteine type is ↓ methionine, ↑ cysteine, ↑B12 and folate in diet
Cystathionine synthase deficiency
382
In this kind of Homocystinuria, Which is the treatment in ↓ Affinity of cystathionine synthase for pyridoxal phosphate?
↑↑ B6 and ↑ cysteine in diet
383
The treatment in this Homocysteine type is just to increase Methionine in diet
Homocysteine methyltransferase (methionine synthase) deficiency
384
What is the result of each type of homocystinuria?
All forms result in excess homocysteine
385
Which findins are expected in homocystinuria?
↑↑ homocysteine in urine, intellectual disability, osteoporosis, tall statur, kyphosis, lens subluxation, thrombosis, and atherosclerosis (stroke and MI)
386
Methionine is the result from homocysteine but which is the enzyme and cofactors that manage it?
Homocysteine methyltransferase and B12
387
Cystathione synthase and vitamin B6 are required for getting Cystathinine, but Which are the substrate required to get it?
Homocysteine and Serine
388
Which is the product of Cystathionine?
Cysteine
389
Which is the explantion of cystinuria?
Hereditary defect of renal PCT and intestinal amino acid transporter for Cysteine, Ornithine, Lysine and Arginie (COLA)
390
Which could be the result of excess cystine in the urine?
Can lead to precipitation of hexagonal cystine stones
391
This is the inheritance mode of cystinuria
Autosomal recessive
392
How common the cystinuria?
Common 1: 7000
393
Which is the diagnostic tool of cystinuria?
Urinary cyanide nitroprusside test is diagnostic
394
Which is the treatment for cystinuria?
Urinary alkalinization and chekating agents increase solubility of cystine stones
Good hydration
395
Which is the mnemonic of glycogen storage disease types?
Very Poor Carbohydrate Metabolism
396
How is made the cystine?
Made of 2 cysteines connected by a disulfide bond
397
Which is the principle of the maple syrup urine disease?
Blocked degradation of branched amino acids due to ↓ α Ketoacid dehydrogenase (B1)
398
What does the Blocked degradation of branched amino acids due to ↓ α Ketoacid dehydrogenase (B1) causes?
Causes ↑ α Ketoacids in blood, especially those of leucine
399
Clinically what does the maple syrup urine disease causes?
Severe CNS defects, intellectual disability and death
400
The maple syrup urine disease is a autosomal dominant or autosomal recessive disease?
Autosomal Recessive
401
Why does the maple syrup urine disease has that name?
Because the urine smells like maple syrup/burnt sugar
402
Which are the three branched aminoacids?
Isoleucine
Leucine
Valine
403
Which are the restriction in diet in the Maple syrup urine disease? and what would you supplement?
Restriction of Leucine, isoleucine and valine in diet
| Supplementation of Thiamine
404
In the glycogen structure how are the branches and linkage composed?
Branches have α- (1,6) bonds; linkages have α-(1,4) bonds
405
These tissues have high reserves of glycogen
Skeletal muscle
| Hepatocytes
406
In the skeletal muscle the glycogen is rapidly metabolized, so which is the process of glycogenolysis
Glycogen → glucose 1 phosphate → Glucose 6 P
407
What realationship does the Glycogen has with the liver?
Glycogen is stored and undergoes glycogenolysis to maintain blood sugar at appropiate levels
408
Which is the process of Glycogenoslysis?
Glycogen phosphorylase cleaves glucose 1 P residues off branched glycogen until four remain befora a branch point. Then 4 α-D- glucanotransferase moves three glucose 1 Ps from the branch to the linkage. Then α- 1,6 glucosidase cleaves off the last glucose 1 P on the branch
409
During the glycogenolysis which are consider debranching enzymes?
4 α-D- glucanotransferase
| α- 1,6 glucosidase
410
A small amount of glycogen is degraded in lysosomes by which enzyme?
α- 1,4 glucosidase (Acid Maltase)
411
How many Glycogen storage diseases exist?
12 types
412
Which are the four most common types of Glycogen storage disease?
```
Von Gierke disease (Type I)
Pompe disease (Type II)
Cori disease (Type III)
McArdle disease (Type V)
```
413
Which is the only X Recessive disease in the Sphingolipidoses?
Fabry disease, The other are Autosomal Recessive
414
What kind of inheritance pattern follows all the glycogen storage disease?
Autosomal recessive
415
What are the Gaucher cells?
Lipid laden macrophages resembling crumpled tissue paper
416
Which enzyme is deficient in Von Gierke disease?
Glucose 6 phosphate
417
Which would be the management of Von Gierke disease?
Frequent oral glucose / cornstarch; avoidance if fructose and galactose
418
This glycogen storage disease has Lysosomal α 1, 4 glucosidase (acid maltase) deficiency
Pompe disease (Type II)
419
Which findings are common in Pompe disease?
Cardiomyopathy and systemic findings leading to early death
420
This is a milder form type I Glycogen storage disease but with normal blood lactate
Cori disease (Type III)
421
How is the gluconeogenesis in Cori disease?
Is intact
422
Which enzyme is related to Cori disease?
α 1,6 glucosidase
423
In which tissue is McArdle disease (Type V) more related? and with which enzyme?
Skeletal muscle glycogen phosphorylase (myophosphorilase)
424
How is the glycogen in the muscle in McArdle disease?
↑ glycogen in muscle, but cannot break it down
425
So... if there is ↑ glycogen in muscle, but cannot be broken down in McArdle disease, which are the clinical manifestations during strenous excercise?
Painful muscle cramps, myoglobinuria (red urine)
426
Which other anormalities can be found in McArdle disease?
Arrythmia from electrolyte abnormalities
427
Name the two type of Lysosomal storage disease
Sphingolipidoses and Mucopolysaccharidoses
428
Which are the shingolipidoses seen in the Lysosomal storage disease?
```
Fabry disease
Gaucher disease
Niemann Pick disease
Tay Sachs disease
Krabbe disease
Metachromatic leukodystrophy
```
429
The findings in this sphingolipidoses is peripheral neuropathy of hands/feet, angiokeratomas, cardivascular/renal disease
Fabry disease
430
Which enzyme is deficient and which substrate is accumulated in Fabry disease?
α galactosidase A deficiency
| Ceramide trihexiside is accumulated
431
Which is the only X Recessive disease in the Sphingolipidoses?
Fabry disease
432
This is the most common lysosomal storage disease and is part of the sphingolipidoses
Gaucher disease
433
What are the Gaucher cells?
Lipid laden macrophages resembling crumpled tissue paper
434
These are the posible findings in Gaucher disease
Hepatosplenomegaly, pancytopenia, aseptic necrosis of Femur, bone crises, Gaucher cells
435
Which enzyme is deficient in Gaucher disease, which also is the treatment...
Glucocerebrosidase (β- glucosidase)
436
In Gaucher disease if Glucocerebrosidase is the deficent enzyme... Who is the accumulated substrate?
Glucocerebroside
437
Progressive neurodegeneration, hepatosplenomegaly, cherry red spot on macula, and foam cells... which lysosomal storage disease is?
Niemann Pick disease
438
What are the foam cells?
Lipid laden macrophages seen in Niemman Pick disease
439
Sphingomyelinase is the deficient enzyme and sphingomyelin the accumulated substrate
Niemann Pick disease
440
Which sphingolipidose disease is clinically similar to Niemann Pick?
Tay Sachs disease
441
Clinically which are the similitudes and difference between Niemman Pick and Tay-Sachs disease?
Similitudes is the progressive neurodegeneration, developmental delay, cherry red spot on macula
Difference, on Tay Sachs there is no hepatosplenomegaly (vs Niemman Pick)
442
Which is the accumulated substrate in Tay Sachs disease?
GM2 ganglioside
443
Hexoaminidase A is the deficient enzyme in this lysosomal storage disease
Tay Sachs disease
444
So... if there is a Carnitine deficiency which would be the expected findings?
Causes weakness, hypotonia, and hypoketotic hypoglicemia
445
This sphingolipidose disease has two accumulated substrates due to Galactocerebrosidase deficiency
Krabbe disease
446
Which are the two accumulated substrates in Krabe disease?
Galactocerebroside, psychosine
447
Peripheral neuropathy, developmental delay, optic atrophy are characteristics of this lysosomal storage disease
Krabbe disease
448
How are the cells in Krabbe disease?
Globoid cells
449
These findings are seen in Metachromatic leukodystrophy
Central and peripheral demyelination with ataxia, dementia
450
Arylsulfatase A is the deficient enzyme on this sphingolipidose disease
Metachromatic leukodystrophy
451
If Arylsulfatase A is the deficient enzyme on Metachromatic leukodystrophy, which is the accumulated substrate
Cerebroside sulfate
452
Hurler and Hunter syndrome what kind of Lysosomal storage diease are they consider?
Mucopolysaccharidoses
453
On this Mucopolysaccharidose disease Heparan sulfate and dermatan sulfate are the substrates accumulated but the deficient enzyme is α L iduronidase
Hurler syndrome
454
Which findings are common in Hurler syndrome?
Developmental delay, gargolysm, airway obstruction, corneal clouding, hepatosplenomegaly
455
Which are the accumulated substrates in both Hurler and Hunter syndrome?
Dermatan sulfate and heparan sulfate
456
Clinically how are diffent Hurler and Hunter syndrome?
Hunter is a mild Hurler + aggressive behavior, no croneal clouding in Hunter disease
457
This is the deficient enzyme in Hunter disease
Iduronate sulfatase
458
Genetically which difference do Hunter and Hurler disease have?
Hunter syndrome is X linked recessive
| Hurler syndrome is Autosomal recessive
459
Which lysosomal storage diseases have an increased incidence in Askenazi Jews?
Tay Sachs, Niemann Pick and some forms of Gaucher disease
460
What does the long chain fatty acid degradation requires?
Carnitine - deoendent transport into the mitochondrial matrix
461
Is β hydroxybutyrate detected in urine test for ketones?
No, is not detected
462
So... if therer is a Carnitine which would be the expected findings?
Causes weakness, hypotonia, and hypoketotic hypoglicemia
463
Who is increased and who is descreased during the Acyl CoA dehydrogenase deficiency?
↑ dicarboxylic acids
| ↓ glucose and ketones
464
What role does Acetyl CoA plays with pyruvate carboxylase?
Acetyl CoA is + allosteric regulator of pyruvate carboxylase in gluconeogenesis
465
Which is the repercussion if ↓ Acetyl CoA?
↓ Fasting Glucose
466
Which substrate is the beginning for the Fatty acid synthesis?
Citrate
467
Where do we find citrate?
Mitochondria matrix
468
Where does the ATP citrate lyase catalyzes citrate in order to have Acetyl CoA?
Cell cytoplasm
469
How many Carbons does palmitate has?
16 C
470
Which are the results of the degradation of Fatty acis?
Ketone bodies
| TCA cycle
471
Fatty acids and aminoacids where are they metabolized?
In the liver
472
Which are metabolized products of Fatty acids and aminoacids?
Acetoacetate and β hydroxybutyrate
473
Which tissues use Acetoacetate and β hydroxybutyrate?
Muscle and brain
474
Which metabolite is depleted in prolonged starvation and diabetic ketoacidosis?
Oxaloacetate
475
During which metabolic process is oxaloacetate depleted in prolonged starvation and diabetic Ketoacidosis?
Gluconeogenesis
476
What happens to oxaloacetate in alcoholism?
Excess NADH shunts oxaloacetate to malate
477
If there is a build up of Acetyl CoA during Prolonged starvation, diabetic ketoacidosis and alcoholism, what is the result?
Acetyl CoA shunts glucose and FFA toward the production of ketone bodies
478
If there is an excess of Ketone bodies, how is it clinically manifested?
Smells like acetone (fruit odor)
479
Is β hydroxybutyrate detected in urine test for ketones?
No, is not detected
480
How many calories per gram does the protein or carbohydrates give?
1g protein or carbohydrate= 4 kcal
481
1 gram of Fat how many calories does is gives?
9 kcal per 1 g fat
482
These calories you get per 1 g of alcohol
1 g alcohol= 7 kcal
483
During excercise the percentage of maximal energy at the begining is 100% but decreases rapidly, which is the first source of energy used that is burned in 2 seconds?
Stored ATP
484
This metabolism manages % of maximal energy for the largest periods
Aerobic metabolism
485
During fasting and starvation which are the priorities?
Are to supply sufficient glucose to the brain and RBCs and to preserve proteins
486
During fed state (after a meal) which metabolic states are present?
Glycolisis and aerobic respiration
487
During fed state (after a meal) what happens to insulin?
Insulin stimulates storage of lypids, protein and glycogen
488
These are the three main metabolic pathways that occur during Fasting (between meals)
Hepatic glycogneolysis
| Hepatic gluconeogenesis and adipose release of FFA
489
Which is the major metabolic event that happens in Fasting?
Hepatic glycogneolysis
490
These are the minor metabolic events that happens in Fasting?
Hepatic gluconeogenesis and adipose release of FFA
491
During fasting, they stimulate use of fuel reserves
Glucagon
| Adrenaline
492
During the 1-3 day of starvation, how are the blood glucose levels mantained?
Hepatic glycogenolysis
Adipose release of FFA
Muscle and liver, which shift fuel use from glucose to FFA
Hepatic gluconeogenesis from peripheral tissue lactate and alanine, and from adipose tissue glycerol and propionyl CoA
493
Which are the only triacylglycerol components that contribute to gluconeogenesis?
From odd chain FFA
494
After starvation in how many days the glycogen reserves are depleted?
After day 1
495
Can the RBCs use ketones?
No, because they lack mitochondria
496
If you were to last 8 weeks of starvation, in which order the source of energy will be depleated?
Carbohydrates depleted in less than 3 days
Fat in 8 weeks
Protein the 8 weeks and more, Vital protein degradation
497
After day 3 of starvation which is the next main source of energy for the brain?
Adipose stores- Ketones bodies become the main source of energy to the brain
498
After the ketones bodies are depleted during starvation, what is next?
Vital protein degradation accelerates , leading to organ failure and death
499
During starvation after day 3 what determines the survival time?
Amount of excess stores
500
In the cholesterol synthesis which enzyme converts HMG- CoA to mevalonate?
HMG- CoA reductase
501
Who induces HMG CoA reductase in the cholesterol synthesis?
Insulin
502
Who esterifies 2/3 of plasma cholesterol?
Lecithin-cholesterol acyltransferase (LCAT)
503
Which is the effect of statins?
Competitively and reversibly inhibit HMG- CoA reductase
504
Who manages the degradation of dietary triglycerides in the small intestine?
Pancreatic lipase
505
What is the function of Lipoprotein lipase (LPL)?
Degradation of Triglycerides circulating in chylomicrons and VLDLs
506
Where can we find Lipoprotein lipase?
Found on vascular endothelial surface
507
Its function is the degradation of Triglycerides remaining in IDL
Hepatic Triglyceride Lipase (HL)
508
Which is the effect of Hormone sensitive lipase?
Degradation of TG stored in adipocytes
509
Catalyzes esterification of cholesterol
Lecitin Cholesterol Acyl Transferase (LCAT)
510
Who mediates transfer of cholesterol esters to other lipoprotein particles?
Cholesterol ester transfer protein (CETP)
511
Which organs produce Nascent HDL
Liver and intestine
512
Which enzyme is required for nascent HDL to become mature HDL?
LCAT- Lecitin Cholesterol Acyl Transferase
513
From Mature HDL which enzyme is required to form the transfer cholesterol esters to VLDL, IDL, LDL?
CETP- Cholesterol ester transfer protein
514
Proteins that bind lipids to form lipoproteins, they transport the lipids through the lymphatic and circulatory system
Apolipoprotein
515
Which apolipoprotein mediates remnant uptake?
Apo E
516
Where can we find ApoE?
In every kind of lipoprotein except LDL
517
What is the function of Apo A I?
Activates LCAT
518
This apolipoprotein is just found in Chylomicron and HDL
Apo A I
519
It´s consider a Lipoprotein lipase cofactor
Apo C II
520
Where do we find Apo C II?
Chylomicron, VLDL and HDL
521
Found in Chylomicrons and chylomicrons remnant and mediates chylomicron secretion
Apo B 48
522
This Apoliporpotein binds LDL receptor
Apo B 100
523
These are the lipoproteins where we can find Apo B 100
VLDL, IDL, LDL
524
Which is the composition of Lipoproteins?
Cholesterol
Triglycerides
Phospholipids
525
Which lipoprotein carry most cholesterol?
HDL and LDL
526
Which is the direction that LDL transports cholesterol?
From liver to Tissues
527
This is the direction that HDL transports cholesterol
Periphery to Liver
528
What is the work for chylomicrons related to the triglycerides?
Delivers dietary triglycerides to peripheral tissue
529
This lipoprotein delivers cholesterol to liver?
Chylomicron remnants
530
This kind of chylomicron is mostly depleted of their triacylglycerols
Chylomicron remnants
531
Who secretes chylomicrons?
Intestinal epithelial cells
532
DElivers hepatic triglycerides to peripheral tissue
VLDL
533
Where is secreted the VLDL?
In the liver
534
Formed in the degradation of VLDL
IDL
535
Which is the function of IDL?
Delivers Triglycerides and cholesterol to liver
536
How does LDL is formed?
Formed by hepatic lipase modification of IDL in the peripheral tissue
537
Delivers hepatic cholesterol to peripheral tissues
LDL
538
How does the target cells take up the LDL?
Via receptor mediated endocytosis
539
Which is the function of HDL?
Mediates reverse cholesterol transport from periphery to liver
540
Acts as a repository for apoC and apoE (which are needed for chylomicron and VLDL metabolism)
HDL
541
Who secretes HDL?
Liver and intestine
542
Which is the relationship between alcohol and HDL?
Alcohol increases HDL synthesis
543
In this familial dyslipidemia type I, Who is increased in the blood level?
Chylomicrons, Tryglicerides, cholesterol
544
Which other name does the Familial dyslipidemia type I receives?
Hyperchylomicronemia
545
What kind of mode of inheritance is seen in Familial dyslipidemia type I?
Autosomal recessive
546
What is the problem in Familial dyslipidemia Hyperchylomicronemia?
Lipoprotein lipase deficiency or altered apolipoprotein C II
547
Which can be the consequences of Familial dyslipidemia type 1?
Pancreatitis , hepatosplenomegaly, and eruptive/ pruritic xanthomas
548
How is the risk of atherosclerosis in Familial dyslipidemia type 1?
No increased risk for atherosclerosis
549
In this kind of familial Dyslipidemia the LDL receptors are abscent or defective
Familial dyslipidemia type IIa
550
Familial hypercholesterolemia also knowns as?
Familial dyslipidemia type IIa
551
What is increased in blood in the Familial hypercholesterolemia?
LDL and cholesterol
552
This is the inheritance mode of Familial dyslipidemia type IIa
Autosomal Dominant
553
Which is the incidence of Familial hypercholesterolemia heterozygote?
1: 500
554
Which are the expected levels of cholesterol seen in an heterozygote Familial hypercholesterolemia patient?
300 mg/ dL
555
The homozygotes Familial hypercholesterolemia patients are very rare, but which levels of cholesterol would you expect to see?
700 mg/ dL
556
How is the risk of atherosclerosis in Familial dyslipidemia type IIa?
Acelerated atherosclerosis (may have MI before 20)
557
Which clinical findings can be seen in Familial dyslipidemia type IIa?
Tendon (Achiles) xanthomas and corneal arcus
558
In this familial dyslipidemia, VLDL and triglycerides are increased in blood levels
Type IV or hypertriglyceridemia
559
Which is the cause of Familial hypertriglyceridemia?
Hepatic overproduction of VLDL
560
Familial dyslipidemia type IV Is it Autosomal Dominant or Autosomal recessive?
Autosomal dominant
561
Which is the possible finding in Familial hypertriglyceridemia?
Pancreatitis
