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Noorda FOM > Module 5 > Flashcards

Module 5 Flashcards

1
Q

Major anabolic hormone that regulates fuel storage

A

Insulin

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2
Q

Major hormone of fuel mobilization

A

Glucagon

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3
Q

Amount of glucose required by the body per day

A

190 g

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4
Q

Amount of glucose required by the brain per day

A

150 g

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5
Q

What 5 specific categories is ATP used for (catabolism)

A

Biosynthesis
Detoxification
Muscle contraction
Active ion transport
Thermogenesis

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6
Q

Daily dietary cholesterol recommendations

A

Less than 300 mg for healthy non-arteriosclerosis individuals
Less than 200 mg for healthy arteriosclerosis individuals

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7
Q

Catabolic reactions generate ___(7)____ from ___(3)___

A

Heat
Energy
ATP
NADH
CO2
Water
Ammonia

Carbs
Fats
Proteins

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8
Q

Anabolic reactions generate ___(4)____ from ___(4)___

A

Proteins
Polysaccharides
Lipids
Nucleic acids

Amino acids
Fatty acids
Sugars
Nitrogenous bases

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9
Q

Respiratory complex I is also known as

A

NADH: ubiquinone oxidoreductase

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10
Q

Respiratory complex III is also known as

A

Cytochrome C reductase

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11
Q

Respiratory complex IV is also known as

A

Cytochrome oxidase

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12
Q

Electron donors in oxidative phosphorylation

A

NADH
H+
FADH2

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13
Q

Electron acceptors in oxidative phosphorylation

A

NAD+
FAD

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14
Q

What does electron acceptor mean

A

Reduced

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15
Q

Source of acetyl-CoA during fasting

A

Fatty acids

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16
Q

Source of acetyl-CoA during eating

A

Glucose
Fructose
Galactose

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17
Q

TCA cycle takes place in

A

Mitochondrial matrix

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18
Q

Coenzyme A and pyruvate form

A

Acetyl-CoA

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19
Q

High levels of acetyl-CoA in the liver lead to

A

Beta-hydroxybutyrate

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20
Q

How does glucose enter cells

A

Na+ and ATP-independent transport system (secondary/facilitated)
Na+ and ATP-dependent co-transport system (active)

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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+

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22
Q

What transporter does fructose use to enter the cell via the Na+ and ATP-dependent co-transport system

A

GLUT-5

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23
Q

What transporter do glucose/galactose/fructose use to enter circulation via the Na+ and ATP-dependent co-transport system

A

GLUT-2

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24
Q

What tissues are SGLT’s found

A

Renal tubules
Intestinal epithelium (apical membrane)

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25
Q

What cells/tissues have GLUT-1 (6)

A

Hepatocytes
Pancreatic beta cells
RBCs
Brain (Blood-brain barrier)
Cornea
Placenta

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26
Q

What tissues are GLUT-2 found

A

Liver
Kidney

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27
Q

What type of transport are SGLT’s

A

ATP-dependent secondary active

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28
Q

What type of transport are GLUT-1

A

Facilitated

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29
Q

What type of transport are GLUT-2

A

Facilitated

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30
Q

Function of SGLT’s (2)

A

Intestine: glucose absorption
Renal tubules: glucose reabsorption

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31
Q

Functions of GLUT-1 (4)

A

Liver - hormone (thyroid) mediated glucosal bi-directional transport
Pancreas - regulate blood glucose levels
RBCs and Blood-brain-barrier - high glucose affinity

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32
Q

Function of GLUT-2

A

Removes excess glucose from blood
Hepatic glucose uptake (Glycolysis) and output (gluconeogenesis), low affinity

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33
Q

What tissues are GLUT-3 found

A

Brain
CNS
Placenta

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34
Q

What tissues are GLUT-4 found

A

Skeletal muscle
Cardiac muscle
Adipocytes

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35
Q

What tissues are GLUT-5 found (2)

A

Small intestine
Testes

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36
Q

What tissue is GLUT-7 found (1)

A

Liver

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37
Q

What type of transport are GLUT-3

A

Facilitated

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38
Q

What type of transport are GLUT-4

A

Facilitated

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39
Q

What type of transport are GLUT-5

A

Facilitated

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40
Q

What type of transport are GLUT-7

A

Facilitated

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41
Q

Function of GLUT-3

A

Basal glucose uptake
High affinity for glucose
Brain glucose homeostasis mainly involves GLUT-3 and GLUT-1

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42
Q

Function of GLUT-4

A

Removes excess of glucose from blood
Expression is regulated by insulin

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43
Q

Function of GLUT-5

A

Fructose transport

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44
Q

Functions of GLUT-7 (2)

A

Intracellular transport in liver
Mediate glucose release from ER coupled to glucose-6-phosphate

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45
Q

Oxaloacetate is broken down via _______ to ______ for ______ (3 of 3)

A

PEP carboxykinase
Phosphoenolpyruvate (PEP)
Gluconeogenesis

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45
Q

Types of LDH (lactate dehydrogenase)

A

Muscle
Heart
Other tissues have mix

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46
Q

Oxaloacetate is broken down via _______ to ______ for ______ (1 of 3)

A

Citrate synthase
Citrate
TCA cycle

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47
Q

Pyruvate is broken down via _______ to ______

A

Pyruvate carboxylase + biotin + ATP
Oxaloacetate

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48
Q

Oxaloacetate is broken down via (enzyme) to (substrate) for (AA) (2 of 3)

A

Aspartate transaminase (AST)
Aspartate
Asparagine

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49
Q

How does pyruvate enter the mitochodria

A

Pyruvate translocase

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49
Q

What are enzyme 1’s (E1) co-enzyme/protein in the pyruvate dehydrogenase complex

A

Pyruvate dehydrogenase
Thiamine pyrophosphate (TPP)

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49
Q

What enzyme turns pyruvate into acetyl-CoA

A

Pyruvate dehydrogenase (PDH)

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50
Q

What is enzyme 2 (E2) and it’s co-enzymes/proteins in the pyruvate dehydrogenase complex

A

Dihydrolipoyl transacetylase
- Lipoamide
- Coenzyme A

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51
Q

What is enzyme 3 (E3) and it’s co-enzymes/proteins in the pyruvate dehydrogenase complex

A

Dihydrolipoyl dehydrogenase
- Flavin adenine dinucleotide (FAD)
- Nicotinamide adenine dinucleotide (NAD+)

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52
Q

ΔG for pyruvate dehydrogenase complex

A

-33.4 kJ/mol

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53
Q

ΔG for pyruvate under anaerobic conditions

A

-25.1 kJ/mol

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54
Q

Diseases due to PDH gene deficiency (5)

A

E1a: x-linked lactic acidosis
E1b: autosomal r. episodic ataxia
E2: autosomal r. cerebral dysgenesis
E3: autosomal r. infantile epilepsy
Leigh syndrome

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55
Q

Symptoms of PDH gene deficiecy

A

Hypotonia
Seizures
Ataxia
Lactic acidosis
Neurological defects
Infants with the prenatal onset form brain malformations; epicanthic folds, flat nasal bridge, long philtrum

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56
Q

What is Leigh syndrome and its causes (3)

A

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

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57
Q

What other name is Leigh syndrome known as

A

Subacute necrotizing encephalomyelopathy

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58
Q

What causes reduced expression of pyruvate dehydrogenase (2)

A

Wernicke-Korsakoff syndrome
Arsenic poisoning

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59
Q

What is the normal value for lactic acid tests

A

4.5 - 19.8 mg/dL

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59
Q

Causes of lactic acidosis (11)

A

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

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59
Q

What causes Wernicke-Korsakoff syndrome

A

Reduction in dietary thiamine pyrophosphate
Reduction in dietary B2, B3, B5

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60
Q

Symptoms of arsenic poisoning

A

Lactic acidosis
Headaches
Confusion
Convulsions
Heart diseases
Squamous cell carcinoma

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60
Q

Symptoms of Wernicke-Korsakoff syndrome

A

Lactic acidosis
Neurological disturbances
Paralysis
Atrophy of limbs
Cardiac failure

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61
Q

Types of lactic acidosis (3)

A

A - Hypoxia/hypoperfusion
B - Non-hypoxia related (1, 2, 3)
D - D-lactose related

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62
Q

Causes of lactic acidosis A (5)

A

Ischemia
Shock
CO poisoning
Respiratory complications
Severe anemia

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62
Q

Cause of lactic acidosis B1 (disease related) (3)

A

Diabetic ketoacidosis
Lymphoma
Vitamin B1 deficiency

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63
Q

Cause of lactic acidosis D

A

Due to carbohydrate and glucose released by bowel bacteria during short bowel syndrome

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64
Q

Causes of lactic acidosis B2 (drugs and chemical related) (7)

A

Biguanides (metformin)
Cyanide
Salicylates
Ethanol/methanol
Valproic acid
HIV drugs
Chemotherapy drugs

65
Q

Cause of lactic acidosis B3 (inborn errors of metabolism)

A

Pyruvate dehydrogenase
Glucose-6-phosphatase (von Geirke disease)
Pyruvate carboxylase

66
Q

Common symptoms of lactic acidosis (9)

A

Headaches
Abdominal pain
Weight loss
Restlessness (malaise)
Nausea and vomiting
Rapid and shallow breathing
(Tachypnea/Dyspnea)
Mental disruption
Increased heart rate
Fatigue

67
Q

Lab results for lactic acidosis

A

Increased serum lactic acid levels
Increased anion gap
Increased blood lactate and pyruvate
Decreased blood pH (Normal = 7.4)

68
Q

Common management of lactic acidosis

A

Thiamine supplementation
Bicarbonate infusion (keep pH above 7.1)

69
Q

Treatment for septic lactic acidosis

A

Antibiotics followed by surgical drainage/debridement

70
Q

Treatment for hypovolemic or cardiac shock (2)

A

Restoration of perfusion
Adequate tissue oxygenation
(CPR)

71
Q

Treatment for asthma/COPD lactic acidosis

A

High dose beta-2 agonists gradually tapered

72
Q

Mutations is spectrin results in abnormal shaped erythrocytes causing (3)

A

Spherocytosis
Elliptocytosis
Pyropoikilocytosis

73
Q

Evolution of stem cell to erythrocyte (6)

A

Stem cell
Proerythroblast
Erythroblast
Normoblast
Reticulocyte
Erythrocyte

74
Q

The erythrocyte shunts/pathways in glycolysis

A

Embden-Meyerhof (regular glycolysis)
Methemoglobin Reductase
Pentose Phosphate
Luebering-Rapoport

75
Q

What is the Embden-Meyerhof pathway for erythrocytes

A

Glucose -> lactose

76
Q

What is the Methemoglobin Reductase pathway for erythrocytes

A

Glucose -> glucose-6-phosphate -> (glyceraldehyde-3-phosphate dehydrogenase) NAD+ -> (cytochrome b5 reductase) cytochrome b5 Fe3+ -> cytochrome b5 Fe2+

77
Q

What is the Pentose Phosphate pathway (HMP shunt) for erythrocytes

A

Glucose -> glucose-6-phosphate -> ribose-5-phosphate

78
Q

What is the Luebering-Rapoport pathway for erythrocytes

A

Glucose —-> 1,3-diphosphoglycerate -> (bisphosphoglycerate mutase) 2,3-diphosphoglycerate (2,3-BPG) -> (bisphosphoglycerate phosphatase) 3-phosphoglycerate

79
Q

Why is 2,3-BPG so important (6)

A

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
Q

What deficiencies cause the 2,3-BPG levels to decrease (5)

A

Hexokinase
Phosphoglucose isomerase
Phosphofructokinase
Aldolase
BPG mutase/phosphatase (RBC mass increases)

81
Q

What deficiencies cause 2,3-BPG levels to increase

A

Pyruvate kinase

82
Q

What are the 3 sources for acetyl-CoA

A

Amino acids - deamination and oxidation
Carbs - glycolysis
Fatty acids - beta-oxidation

83
Q

What is the first step in TCA cycle

A

Condensation of oxaloacetate with acetyl-CoA to citrate via [citrate synthase]

84
Q

Step 2 of TCA cycle

A

Isomerization of citrate to isocitrate via [aconitase]

85
Q

Step 3 of TCA cycle

A

Oxidative decarboxylation of isocitrate to alpha-ketoglutarate via [isocitrate dehydrogenase]

86
Q

Step 4 of TCA cycle

A

Oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA via [alpha-ketoglutarate dehydrogenase]

87
Q

Step 5 of TCA cycle
(Rxn type, substrate, product, enzyme)

A

Substrate level phosphorylation of succinyl-CoA to succinate via [succinyl-CoA synthetase]

88
Q

Step 6 of TCA cycle

A

Dehydrogenation of succinate to fumarate via [succinate dehydrogenase]

89
Q

Step 7 of TCA cycle

A

Hydration of fumarate to malate via [fumarase]

90
Q

Step 8 of TCA cycle

A

Dehydrogenation of malate to oxaloacetate via [malate dehydrogenase]

91
Q

What regulates TCA cycle (3)

A

Substrate availability
Product levels
Competitive feedback inhibition

92
Q

Products of TCA cycle

A

10 - 12 ATP/acetyl-CoA

93
Q

Where does succinate dehydrogenase originate

A

Inner membrane of mitochondria

94
Q

Where is the PDH complex located

A

Mitochondrial matrix

95
Q

TCA cycle linked diseases for thiamine (Vitamin B1) (3)

A

Beriberi
Nerve degeneration
Lactic acidosis

96
Q

TCA cycle linked deficiencies for riboflavin (Vitamin B2) (2)

A

Developmental abnormalities
Growth retardation

96
Q

TCA cycle linked deficiencies for Niacin (Vitamin B3) (3)

A

Pellagra
Dermatitis
Muscle fatigue

97
Q

TCA cycle linked deficiencies for pantothenate (Vitamin B5) (4)

A

Fatigue
Retorted growth
Anemia
Cramps

98
Q

What enzymes are affected by all vitamin B deficiencies

A

PDH (pyruvate dehydrogenase)
KDH (alpha-ketoglutarate dehydrogenase)

99
Q

What enzyme is affected by riboflavin (B2) deficiency

A

SDH (succinate dehydrogenase)

100
Q

What enzyme is affected by niacin (B3) deficiency

A

MDH (malate dehydrogenase)

101
Q

Prosthetic class vitamins

A

B1 thiamine
B2 riboflavin

102
Q

Co-substrate class vitamins

A

B3 niacin
B5 pantothenate

103
Q

Thiamine (B1) coenzyme

A

TPP (thiamine pyrophosphate)

104
Q

Riboflavin (B2) coenzyme

A

FAD

105
Q

Niacin (B3) coenzyme

A

NAD

106
Q

Pantothenate (B5) coenzyme

A

Co-A

107
Q

Alpha-ketoglutarate dehydrogenase gene deficiency disease causes (2)

A

Autosomal recessive
E1 deficiency - 7p13 - 14
E2 deficiency - 14q24.3

108
Q

Symptoms of alpha-ketoglutarate dehydrogenase gene deficiency (8)

A

Developmental delay
Hypotonia
Ataxia
Seizures
Extrapyramidal dysfunction
Elevated urine alpha-ketoglutarate
Decreased beta-hydroxybutyrate to acetoacetate ratio

109
Q

Fumarate gene deficiency disease cause

A

Autosomal recessive
1q42.1

110
Q

Symptoms of fumarate gene deficiency disease (4)

A

Abnormally small head
Abnormal brain structure
Developmental delay
Hypotonia

111
Q

Succinate dehydrogenase gene deficiency cause

A

Homo/heterozygous mutation in SDH subunits

112
Q

What does SDH connect

A

TCA with ETC

113
Q

Succinate dehydrogenase gene deficiency symptoms (8)

A

A - Leigh syndrome
Hypertrophic cardiomyopathy
Ataxia
Cerebral ataxia
Optic atrophy
Renal cell carcinoma
B/C/D - paraganglioma
B/D - Pheochromocytoma

114
Q

Succinyl-CoA synthetase gene deficiency cause

A

Homo/heterozygous mutations in SUCLA1/2 subunits (affects TCA cycle)

115
Q

Succinyl-CoA synthetase gene deficiency symptoms (4)

A

Encephalomyopathy
Developmental delay
Dystonia
Lactic acidosis

116
Q

Malate dehydrogenase gene deficiency cause

A

Mutations in MDH1/2

117
Q

Malate dehydrogenase gene deficiency symptoms (5)

A

Early-onset encephalopathy
Hypotonia
Psychomotor delay
Refractory epilepsy
Lactic acidosis

118
Q

3 chemical poisons to TCA cycle

A

Fluoroacetate
Arsenate
Malonate

119
Q

TCA enzyme affected by fluoroacetate

A

Aconitase

120
Q

TCA enzyme affected by arsenate

A

KDH

121
Q

TCA enzyme affected by malonate

A

SDH

122
Q

Symptoms of fluoroacetate poisoning (6)

A

Abdominal pain
Sweating
Confusion
Agitation
Muscle twitching/seizures
Hypotension

123
Q

What is located on the inner mitochondrial membrane

A

NADH Dehydrogenase
SDH
Cytochrome C-reductase
Cytochrome C-oxidase
ATP synthase of ETC

123
Q

Mitochondrial diseases (3)

A

LHON (Leber hereditary optic neuropathy)
MELAS (mitochondrial encephalopathy, lactic acidosis, and stroke)
MERRF (myoclonic epilepsy with ragged red fibers)

123
Q

Symptoms of malonate poisoning (4)

A

Decreased cellular respiration
Hypertrophic cardiomyopathy
Ataxia
Cerebral ataxia

123
Q

Symptoms of arsenate poisoning (7)

A

Intense abdominal pain
Swelling
Salivation
Vomiting
Diarrhea
Staggering
Death

124
Q

Complex I of ETC contains (2)

A

Flavin mononucleotide
Iron sulfur centers (FeS)

124
Q

What do the heme groups of complex IV contain instead of iron

A

Copper

124
Q

Complex III of ETC contains (2)

A

Cytochrome b
Cytochrome c1

124
Q

Uncoupling agents

A

Thermogenin
Aspirin

125
Q

Complex IV of ETC contains (2)

A

Cytochrome a
Cytochrome a3
(Cytochrome oxidase)

125
Q

What is uncoupling in ETC

A

When ionophores are created in the inner mitochondrial membrane that shortcut F0 on complex V to allow protons back into the matrix

125
Q

Enzyme responsible for phosphorylation step of oxidative phosphorylation

A

ATP synthase (F1 from complex V of ETC)

125
Q

Inhibitors of complex IV of ETC (3)

A

CO
Cyanide
Azide

126
Q

Inhibitor of coenzyme Q (ubiquitin)

A

Statins

126
Q

Inhibitors of complex V of ETC

A

Oligomycin (toxic)

126
Q

Inhibitor of complex II of ETC

A

Malonate

126
Q

Inhibitors of complex I of ETC (3)

A

Barbiturates (GABA agonists)
Rotenone
Biguanide (metformin)

127
Q

Inhibitor of complex III of ETC

A

Antimycin A (toxic)

128
Q

What does electron donor mean

A

Oxidized

129
Q

Rate limiting step of glycolysis

A

Phosphofructokinase irreversibly catalyzes fructose-6-phosphate to fructose-1,6-bisphosphate

130
Q

Rate limiting step of TCA cycle

A

Isocitrate dehydrogenase irreversibly catalyzes isocitrate to alpha-ketoglutarate

131
Q

Irreversible reactions in TCA cycle (3)

A

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
Q

Inhibitors of TCA (2)

A

ATP
NADH

133
Q

Stimulator of TCA

A

ADP

134
Q

Primary sites of gluconeogenesis (3)

A

Liver (initial and primary)
Renal cortex (prolonged)
Intestinal epithelium (minor)

135
Q

What tissue does not participate in gluconeogenesis and why

A

Skeletal muscle
No G-6-Pase

135
Q

Key enzymes in gluconeogenesis (4)

A

Pyruvate carboxylase
Phosphoenolpyruvate carboxykinase
Fructose-1,6-bisphosphatase
Glucose-6-phosphatase

136
Q

Function of Pyruvate carboxylase

A

Pyruvate to oxaloacetate

136
Q

Substrates of gluconeogenesis (4)

A

Glucogenic AA (mainly A, Q)
Lactate
Glycerol
Succinyl-CoA (propionyl-CoA)

136
Q

Function of Glucose-6-phosphatase

A

G-6-P to glucose
Release into bloodstream

137
Q

Function of Fructose-1,6-bisphosphatase

A

F-1,6-P to F-6-P
Rate limiting enzyme

137
Q

Function of Phosphoenolpyruvate carboxykinase

A

Oxaloacetate to phosphoenolpyruvate

138
Q

Rate limiting step of gluconeogenesis

A

F-1,6-P to F-6-P

139
Q

Stimulators of gluconeogenesis (3)

A

Low ATP
Glucagon
Cortisol

140
Q

Inhibitors of gluconeogenesis (4)

A

High ATP
Insulin
ADP
F-2,6-P

141
Q

Type I GSD (Von Gierke) deficiency (2)

A

G-6-Pase (type Ia)
G-6-Translocase (type Ib

142
Q

Symptoms and features of Type I GSD (Von Gierke) (7)

A

Severe fasting hypoglycemia Hepato/renomegaly
Lactic acidosis
Hyperlipidemia
Hyperuricemia
Doll-like face
Anemia

143
Q

Genetic cause for Type I GSD (Von Gierke)

A

Autosomal r. G6PC gene (17q)

144
Q

Type II GSD (Pompe) deficiency

A

Lysosomal acid alpha-glucoside (acid maltase)

145
Q

Symptoms and features of Type II (Pompe) (5)

A

Hypertrophic cardiomyopathy
Macroglossia
Muscle weakness leading to respiratory insufficiency
Proximal myopathy
Intracranial aneurysms

146
Q

Genetic cause for Type II GSD (Pompe)

A

Autosomal r. GAA gene (17q)

147
Q

Type III GSD (Cori) deficiency (2)

A

alpha-1,6-glucosidase
4-alpha-D-glucanotransferase

148
Q

Symptoms and features of Type III (Cori)

A

Muscles
-Weakness
-Cramps
-Cardiomyopathy
Liver
-Hepatomegaly
-Mild hypoglycemia
-Hyperlipidemia

149
Q

Genetic cause for Type II GSD

A

Mutation of AGL on 1p

150
Q

Type V GSD (McArdle) deficiency

A

Glycogen phosphorylase (Myophosphorylase)

151
Q

Symptoms and features of Type V (McArdle)

A

Exercise Intolerance
Second Wind Phenomenon
Rhabdomyolysis leading to myoglobinuria (dark urine)
Electrolyte imbalances
Potential cardiac arrhythmias
Normal serum glucose

152
Q

Genetic cause for Type V GSD (McArdle)

A

Noorda FOM (18 decks)

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