Module 5 Flashcards
(176 cards)
1
Q
Major anabolic hormone that regulates fuel storage
A
Insulin
2
Q
Major hormone of fuel mobilization
A
Glucagon
3
Q
Amount of glucose required by the body per day
A
190 g
4
Q
Amount of glucose required by the brain per day
A
150 g
5
Q
What 5 specific categories is ATP used for (catabolism)
A
Biosynthesis
Detoxification
Muscle contraction
Active ion transport
Thermogenesis
6
Q
Daily dietary cholesterol recommendations
A
Less than 300 mg for healthy non-arteriosclerosis individuals
Less than 200 mg for healthy arteriosclerosis individuals
7
Q
Catabolic reactions generate ___(7)____ from ___(3)___
A
Heat
Energy
ATP
NADH
CO2
Water
Ammonia
Carbs
Fats
Proteins
8
Q
Anabolic reactions generate ___(4)____ from ___(4)___
A
Proteins
Polysaccharides
Lipids
Nucleic acids
Amino acids
Fatty acids
Sugars
Nitrogenous bases
9
Q
Respiratory complex I is also known as
A
NADH: ubiquinone oxidoreductase
10
Q
Respiratory complex III is also known as
A
Cytochrome C reductase
11
Q
Respiratory complex IV is also known as
A
Cytochrome oxidase
12
Q
Electron donors in oxidative phosphorylation
A
NADH
H+
FADH2
13
Q
Electron acceptors in oxidative phosphorylation
A
NAD+
FAD
14
Q
What does electron acceptor mean
A
Reduced
15
Q
Source of acetyl-CoA during fasting
A
Fatty acids
16
Q
Source of acetyl-CoA during eating
A
Glucose
Fructose
Galactose
17
Q
TCA cycle takes place in
A
Mitochondrial matrix
18
Q
Coenzyme A and pyruvate form
A
Acetyl-CoA
19
Q
High levels of acetyl-CoA in the liver lead to
A
Beta-hydroxybutyrate
20
Q
How does glucose enter cells
A
Na+ and ATP-independent transport system (secondary/facilitated)
Na+ and ATP-dependent co-transport system (active)
21
Q
What transporter do glucose/galactose use to enter the cell via the Na+ and ATP-dependent co-transport system, and what cofactor is utilized
A
SGLT-1with Na+
22
Q
What transporter does fructose use to enter the cell via the Na+ and ATP-dependent co-transport system
A
GLUT-5
23
Q
What transporter do glucose/galactose/fructose use to enter circulation via the Na+ and ATP-dependent co-transport system
A
GLUT-2
24
Q
What tissues are SGLT’s found
A
Renal tubules
Intestinal epithelium (apical membrane)
25
What cells/tissues have GLUT-1 (6)
Hepatocytes
Pancreatic beta cells
RBCs
Brain (Blood-brain barrier)
Cornea
Placenta
26
What tissues are GLUT-2 found
Liver
Kidney
27
What type of transport are SGLT's
ATP-dependent secondary active
28
What type of transport are GLUT-1
Facilitated
29
What type of transport are GLUT-2
Facilitated
30
Function of SGLT's (2)
Intestine: glucose absorption
Renal tubules: glucose reabsorption
31
Functions of GLUT-1 (4)
Liver - hormone (thyroid) mediated glucosal bi-directional transport
Pancreas - regulate blood glucose levels
RBCs and Blood-brain-barrier - high glucose affinity
32
Function of GLUT-2
Removes excess glucose from blood
Hepatic glucose uptake (Glycolysis) and output (gluconeogenesis), low affinity
33
What tissues are GLUT-3 found
Brain
CNS
Placenta
34
What tissues are GLUT-4 found
Skeletal muscle
Cardiac muscle
Adipocytes
35
What tissues are GLUT-5 found (2)
Small intestine
Testes
36
What tissue is GLUT-7 found (1)
Liver
37
What type of transport are GLUT-3
Facilitated
38
What type of transport are GLUT-4
Facilitated
39
What type of transport are GLUT-5
Facilitated
40
What type of transport are GLUT-7
Facilitated
41
Function of GLUT-3
Basal glucose uptake
High affinity for glucose
Brain glucose homeostasis mainly involves GLUT-3 and GLUT-1
42
Function of GLUT-4
Removes excess of glucose from blood
Expression is regulated by insulin
43
Function of GLUT-5
Fructose transport
44
Functions of GLUT-7 (2)
Intracellular transport in liver
Mediate glucose release from ER coupled to glucose-6-phosphate
45
Oxaloacetate is broken down via _______ to ______ for ______ (3 of 3)
PEP carboxykinase
Phosphoenolpyruvate (PEP)
Gluconeogenesis
45
Types of LDH (lactate dehydrogenase)
Muscle
Heart
Other tissues have mix
46
Oxaloacetate is broken down via _______ to ______ for ______ (1 of 3)
Citrate synthase
Citrate
TCA cycle
47
Pyruvate is broken down via _______ to ______
Pyruvate carboxylase + biotin + ATP
Oxaloacetate
48
Oxaloacetate is broken down via (enzyme) to (substrate) for (AA) (2 of 3)
Aspartate transaminase (AST)
Aspartate
Asparagine
49
How does pyruvate enter the mitochodria
Pyruvate translocase
49
What are enzyme 1's (E1) co-enzyme/protein in the pyruvate dehydrogenase complex
Pyruvate dehydrogenase
Thiamine pyrophosphate (TPP)
49
What enzyme turns pyruvate into acetyl-CoA
Pyruvate dehydrogenase (PDH)
50
What is enzyme 2 (E2) and it's co-enzymes/proteins in the pyruvate dehydrogenase complex
Dihydrolipoyl transacetylase
- Lipoamide
- Coenzyme A
51
What is enzyme 3 (E3) and it's co-enzymes/proteins in the pyruvate dehydrogenase complex
Dihydrolipoyl dehydrogenase
- Flavin adenine dinucleotide (FAD)
- Nicotinamide adenine dinucleotide (NAD+)
52
ΔG for pyruvate dehydrogenase complex
-33.4 kJ/mol
53
ΔG for pyruvate under anaerobic conditions
-25.1 kJ/mol
54
Diseases due to PDH gene deficiency (5)
E1a: x-linked lactic acidosis
E1b: autosomal r. episodic ataxia
E2: autosomal r. cerebral dysgenesis
E3: autosomal r. infantile epilepsy
Leigh syndrome
55
Symptoms of PDH gene deficiecy
Hypotonia
Seizures
Ataxia
Lactic acidosis
Neurological defects
Infants with the prenatal onset form brain malformations; epicanthic folds, flat nasal bridge, long philtrum
56
What is Leigh syndrome and its causes (3)
A rare, progressive, neurodegenerative, autosomal recessive disorder caused by defects in mitochondrial ATP production
Mutations in genes that encode proteins of the PDH (PDH phosphatase and E1), the ETC, or ATP synthase
57
What other name is Leigh syndrome known as
Subacute necrotizing encephalomyelopathy
58
What causes reduced expression of pyruvate dehydrogenase (2)
Wernicke-Korsakoff syndrome
Arsenic poisoning
59
What is the normal value for lactic acid tests
4.5 - 19.8 mg/dL
59
Causes of lactic acidosis (11)
Hypoxia in tissues
Vigorous physical activity/exercise
Drug-induced
Cardio-respiratory arrest
Neoplastic/Cancer diseases
Toxins
CO poisoning
Sepsis
Thiamine deficiency
PDH deficiency
Mitochondrial respiratory chain failure
59
What causes Wernicke-Korsakoff syndrome
Reduction in dietary thiamine pyrophosphate
Reduction in dietary B2, B3, B5
60
Symptoms of arsenic poisoning
Lactic acidosis
Headaches
Confusion
Convulsions
Heart diseases
Squamous cell carcinoma
60
Symptoms of Wernicke-Korsakoff syndrome
Lactic acidosis
Neurological disturbances
Paralysis
Atrophy of limbs
Cardiac failure
61
Types of lactic acidosis (3)
A - Hypoxia/hypoperfusion
B - Non-hypoxia related (1, 2, 3)
D - D-lactose related
62
Causes of lactic acidosis A (5)
Ischemia
Shock
CO poisoning
Respiratory complications
Severe anemia
62
Cause of lactic acidosis B1 (disease related) (3)
Diabetic ketoacidosis
Lymphoma
Vitamin B1 deficiency
63
Cause of lactic acidosis D
Due to carbohydrate and glucose released by bowel bacteria during short bowel syndrome
64
Causes of lactic acidosis B2 (drugs and chemical related) (7)
Biguanides (metformin)
Cyanide
Salicylates
Ethanol/methanol
Valproic acid
HIV drugs
Chemotherapy drugs
65
Cause of lactic acidosis B3 (inborn errors of metabolism)
Pyruvate dehydrogenase
Glucose-6-phosphatase (von Geirke disease)
Pyruvate carboxylase
66
Common symptoms of lactic acidosis (9)
Headaches
Abdominal pain
Weight loss
Restlessness (malaise)
Nausea and vomiting
Rapid and shallow breathing
(Tachypnea/Dyspnea)
Mental disruption
Increased heart rate
Fatigue
67
Lab results for lactic acidosis
Increased serum lactic acid levels
Increased anion gap
Increased blood lactate and pyruvate
Decreased blood pH (Normal = 7.4)
68
Common management of lactic acidosis
Thiamine supplementation
Bicarbonate infusion (keep pH above 7.1)
69
Treatment for septic lactic acidosis
Antibiotics followed by surgical drainage/debridement
70
Treatment for hypovolemic or cardiac shock (2)
Restoration of perfusion
Adequate tissue oxygenation
(CPR)
71
Treatment for asthma/COPD lactic acidosis
High dose beta-2 agonists gradually tapered
72
Mutations is spectrin results in abnormal shaped erythrocytes causing (3)
Spherocytosis
Elliptocytosis
Pyropoikilocytosis
73
Evolution of stem cell to erythrocyte (6)
Stem cell
Proerythroblast
Erythroblast
Normoblast
Reticulocyte
Erythrocyte
74
The erythrocyte shunts/pathways in glycolysis
Embden-Meyerhof (regular glycolysis)
Methemoglobin Reductase
Pentose Phosphate
Luebering-Rapoport
75
What is the Embden-Meyerhof pathway for erythrocytes
Glucose -> lactose
76
What is the Methemoglobin Reductase pathway for erythrocytes
Glucose -> glucose-6-phosphate -> (glyceraldehyde-3-phosphate dehydrogenase) NAD+ -> (cytochrome b5 reductase) cytochrome b5 Fe3+ -> cytochrome b5 Fe2+
77
What is the Pentose Phosphate pathway (HMP shunt) for erythrocytes
Glucose -> glucose-6-phosphate -> ribose-5-phosphate
78
What is the Luebering-Rapoport pathway for erythrocytes
Glucose ----> 1,3-diphosphoglycerate -> (bisphosphoglycerate mutase) 2,3-diphosphoglycerate (2,3-BPG) -> (bisphosphoglycerate phosphatase) 3-phosphoglycerate
79
Why is 2,3-BPG so important (6)
Doesn't require ATP for glycolysis
Lowers deoxyhemoglobin affinity for oxygen
Helps high altitude adaptation
Facilitates placental oxygen from mother to fetus
Levels rise in anemic and COPD patients
80
What deficiencies cause the 2,3-BPG levels to decrease (5)
Hexokinase
Phosphoglucose isomerase
Phosphofructokinase
Aldolase
BPG mutase/phosphatase (RBC mass increases)
81
What deficiencies cause 2,3-BPG levels to increase
Pyruvate kinase
82
What are the 3 sources for acetyl-CoA
Amino acids - deamination and oxidation
Carbs - glycolysis
Fatty acids - beta-oxidation
83
What is the first step in TCA cycle
Condensation of oxaloacetate with acetyl-CoA to citrate via [citrate synthase]
84
Step 2 of TCA cycle
Isomerization of citrate to isocitrate via [aconitase]
85
Step 3 of TCA cycle
Oxidative decarboxylation of isocitrate to alpha-ketoglutarate via [isocitrate dehydrogenase]
86
Step 4 of TCA cycle
Oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA via [alpha-ketoglutarate dehydrogenase]
87
Step 5 of TCA cycle
(Rxn type, substrate, product, enzyme)
Substrate level phosphorylation of succinyl-CoA to succinate via [succinyl-CoA synthetase]
88
Step 6 of TCA cycle
Dehydrogenation of succinate to fumarate via [succinate dehydrogenase]
89
Step 7 of TCA cycle
Hydration of fumarate to malate via [fumarase]
90
Step 8 of TCA cycle
Dehydrogenation of malate to oxaloacetate via [malate dehydrogenase]
91
What regulates TCA cycle (3)
Substrate availability
Product levels
Competitive feedback inhibition
92
Products of TCA cycle
10 - 12 ATP/acetyl-CoA
93
Where does succinate dehydrogenase originate
Inner membrane of mitochondria
94
Where is the PDH complex located
Mitochondrial matrix
95
TCA cycle linked diseases for thiamine (Vitamin B1) (3)
Beriberi
Nerve degeneration
Lactic acidosis
96
TCA cycle linked deficiencies for riboflavin (Vitamin B2) (2)
Developmental abnormalities
Growth retardation
96
TCA cycle linked deficiencies for Niacin (Vitamin B3) (3)
Pellagra
Dermatitis
Muscle fatigue
97
TCA cycle linked deficiencies for pantothenate (Vitamin B5) (4)
Fatigue
Retorted growth
Anemia
Cramps
98
What enzymes are affected by all vitamin B deficiencies
PDH (pyruvate dehydrogenase)
KDH (alpha-ketoglutarate dehydrogenase)
99
What enzyme is affected by riboflavin (B2) deficiency
SDH (succinate dehydrogenase)
100
What enzyme is affected by niacin (B3) deficiency
MDH (malate dehydrogenase)
101
Prosthetic class vitamins
B1 thiamine
B2 riboflavin
102
Co-substrate class vitamins
B3 niacin
B5 pantothenate
103
Thiamine (B1) coenzyme
TPP (thiamine pyrophosphate)
104
Riboflavin (B2) coenzyme
FAD
105
Niacin (B3) coenzyme
NAD
106
Pantothenate (B5) coenzyme
Co-A
107
Alpha-ketoglutarate dehydrogenase gene deficiency disease causes (2)
Autosomal recessive
E1 deficiency - 7p13 - 14
E2 deficiency - 14q24.3
108
Symptoms of alpha-ketoglutarate dehydrogenase gene deficiency (8)
Developmental delay
Hypotonia
Ataxia
Seizures
Extrapyramidal dysfunction
Elevated urine alpha-ketoglutarate
Decreased beta-hydroxybutyrate to acetoacetate ratio
109
Fumarate gene deficiency disease cause
Autosomal recessive
1q42.1
110
Symptoms of fumarate gene deficiency disease (4)
Abnormally small head
Abnormal brain structure
Developmental delay
Hypotonia
111
Succinate dehydrogenase gene deficiency cause
Homo/heterozygous mutation in SDH subunits
112
What does SDH connect
TCA with ETC
113
Succinate dehydrogenase gene deficiency symptoms (8)
A - Leigh syndrome
Hypertrophic cardiomyopathy
Ataxia
Cerebral ataxia
Optic atrophy
Renal cell carcinoma
B/C/D - paraganglioma
B/D - Pheochromocytoma
114
Succinyl-CoA synthetase gene deficiency cause
Homo/heterozygous mutations in SUCLA1/2 subunits (affects TCA cycle)
115
Succinyl-CoA synthetase gene deficiency symptoms (4)
Encephalomyopathy
Developmental delay
Dystonia
Lactic acidosis
116
Malate dehydrogenase gene deficiency cause
Mutations in MDH1/2
117
Malate dehydrogenase gene deficiency symptoms (5)
Early-onset encephalopathy
Hypotonia
Psychomotor delay
Refractory epilepsy
Lactic acidosis
118
3 chemical poisons to TCA cycle
Fluoroacetate
Arsenate
Malonate
119
TCA enzyme affected by fluoroacetate
Aconitase
120
TCA enzyme affected by arsenate
KDH
121
TCA enzyme affected by malonate
SDH
122
Symptoms of fluoroacetate poisoning (6)
Abdominal pain
Sweating
Confusion
Agitation
Muscle twitching/seizures
Hypotension
123
What is located on the inner mitochondrial membrane
NADH Dehydrogenase
SDH
Cytochrome C-reductase
Cytochrome C-oxidase
ATP synthase of ETC
123
Mitochondrial diseases (3)
LHON (Leber hereditary optic neuropathy)
MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke)
MERRF (myoclonic epilepsy with ragged red fibers)
123
Symptoms of malonate poisoning (4)
Decreased cellular respiration
Hypertrophic cardiomyopathy
Ataxia
Cerebral ataxia
123
Symptoms of arsenate poisoning (7)
Intense abdominal pain
Swelling
Salivation
Vomiting
Diarrhea
Staggering
Death
124
Complex I of ETC contains (2)
Flavin mononucleotide
Iron sulfur centers (FeS)
124
What do the heme groups of complex IV contain instead of iron
Copper
124
Complex III of ETC contains (2)
Cytochrome b
Cytochrome c1
124
Uncoupling agents
Thermogenin
Aspirin
125
Complex IV of ETC contains (2)
Cytochrome a
Cytochrome a3
(Cytochrome oxidase)
125
What is uncoupling in ETC
When ionophores are created in the inner mitochondrial membrane that shortcut F0 on complex V to allow protons back into the matrix
125
Enzyme responsible for phosphorylation step of oxidative phosphorylation
ATP synthase (F1 from complex V of ETC)
125
Inhibitors of complex IV of ETC (3)
CO
Cyanide
Azide
126
Inhibitor of coenzyme Q (ubiquitin)
Statins
126
Inhibitors of complex V of ETC
Oligomycin (toxic)
126
Inhibitor of complex II of ETC
Malonate
126
Inhibitors of complex I of ETC (3)
Barbiturates (GABA agonists)
Rotenone
Biguanide (metformin)
127
Inhibitor of complex III of ETC
Antimycin A (toxic)
128
What does electron donor mean
Oxidized
129
Rate limiting step of glycolysis
Phosphofructokinase irreversibly catalyzes fructose-6-phosphate to fructose-1,6-bisphosphate
130
Rate limiting step of TCA cycle
Isocitrate dehydrogenase irreversibly catalyzes isocitrate to alpha-ketoglutarate
131
Irreversible reactions in TCA cycle (3)
Acetyl-CoA + oxaloacetate to citrate by citrate synthase condensation reaction
Isocitrate to alpha-ketoglutarate by isocitrate dehydrogenase oxidative decarboxylation reaction
Alpha-ketoglutarate to succinyl-CoA by alpha-ketoglutarate dehydrogenase oxidative dehydrogenase reaction
132
Inhibitors of TCA (2)
ATP
NADH
133
Stimulator of TCA
ADP
134
Primary sites of gluconeogenesis (3)
Liver (initial and primary)
Renal cortex (prolonged)
Intestinal epithelium (minor)
135
What tissue does not participate in gluconeogenesis and why
Skeletal muscle
No G-6-Pase
135
Key enzymes in gluconeogenesis (4)
Pyruvate carboxylase
Phosphoenolpyruvate carboxykinase
Fructose-1,6-bisphosphatase
Glucose-6-phosphatase
136
Function of Pyruvate carboxylase
Pyruvate to oxaloacetate
136
Substrates of gluconeogenesis (4)
Glucogenic AA (mainly A, Q)
Lactate
Glycerol
Succinyl-CoA (propionyl-CoA)
136
Function of Glucose-6-phosphatase
G-6-P to glucose
Release into bloodstream
137
Function of Fructose-1,6-bisphosphatase
F-1,6-P to F-6-P
Rate limiting enzyme
137
Function of Phosphoenolpyruvate carboxykinase
Oxaloacetate to phosphoenolpyruvate
138
Rate limiting step of gluconeogenesis
F-1,6-P to F-6-P
139
Stimulators of gluconeogenesis (3)
Low ATP
Glucagon
Cortisol
140
Inhibitors of gluconeogenesis (4)
High ATP
Insulin
ADP
F-2,6-P
141
Type I GSD (Von Gierke) deficiency (2)
G-6-Pase (type Ia)
G-6-Translocase (type Ib
142
Symptoms and features of Type I GSD (Von Gierke) (7)
Severe fasting hypoglycemia Hepato/renomegaly
Lactic acidosis
Hyperlipidemia
Hyperuricemia
Doll-like face
Anemia
143
Genetic cause for Type I GSD (Von Gierke)
Autosomal r. G6PC gene (17q)
144
Type II GSD (Pompe) deficiency
Lysosomal acid alpha-glucoside (acid maltase)
145
Symptoms and features of Type II (Pompe) (5)
Hypertrophic cardiomyopathy
Macroglossia
Muscle weakness leading to respiratory insufficiency
Proximal myopathy
Intracranial aneurysms
146
Genetic cause for Type II GSD (Pompe)
Autosomal r. GAA gene (17q)
147
Type III GSD (Cori) deficiency (2)
alpha-1,6-glucosidase
4-alpha-D-glucanotransferase
148
Symptoms and features of Type III (Cori)
Muscles
-Weakness
-Cramps
-Cardiomyopathy
Liver
-Hepatomegaly
-Mild hypoglycemia
-Hyperlipidemia
149
Genetic cause for Type II GSD
Mutation of AGL on 1p
150
Type V GSD (McArdle) deficiency
Glycogen phosphorylase (Myophosphorylase)
151
Symptoms and features of Type V (McArdle)
Exercise Intolerance
Second Wind Phenomenon
Rhabdomyolysis leading to myoglobinuria (dark urine)
Electrolyte imbalances
Potential cardiac arrhythmias
Normal serum glucose
152
Genetic cause for Type V GSD (McArdle)
