Module 5-6 Review Flashcards

1
Q

Fed state fuel for the liver
(Fuel,Transport; Pathways (2))

A

Glucose (GLUT2; glycogenesis, lipogenesis)

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2
Q

Fasted state fuel for liver

A

Glucose via glycogenolysis/gluconeogenesis
NO KETONE BODIES

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3
Q

Fed state fuel and function of adipose tissue

A

Glucose
Lipogenesis/fatty acid storage

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4
Q

Fasting state fuel for adipose

A

Free fatty acids via lipolysis

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5
Q

Fed state fuel for muscle

A

Glucose (GLUT4; glycogenesis)

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6
Q

Fasting state fuel for muscle

A

Fatty acids (oxidation), amino acids (prolonged fasting)

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7
Q

Fed state fuel for brain and what are the membrane uptake proteins

A

Glucose (GLUT3 and GLUT1)

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8
Q

Fasting state fuel for brain

A

Glucose, ketone bodies (during prolonged fasting)

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9
Q

Fed state fuel for heart

A

Fatty acids (circulating lipids)

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10
Q

Fasting state fuel for heart

A

Glucose, lactate, pyruvate. Prolonged fasting:
ketone bodies

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11
Q

Fed state fuel for RBC

A

Glucose (GLUT2)

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12
Q

Fasting state fuel for RBC

A

Glucose (hepatic glycogenolysis; gluconeogenesis

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13
Q

What does caffeine inhibit

A

cAMP phosphodiesterase, resulting in PKA activation and glucagon and epinephrine enhanced response

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14
Q

Glucagon additional effects

A

Promote gluconeogenesis by increasing PKA, which decreases f-2,6-bisphosphate and activates f-1,6-bisphosphate and glycogenolysis in the liver

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15
Q

Which diabetes type will likely show ketoacidosis

A

Type 1

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16
Q

Symptoms and treatment of ketoacidosis

A

Polyuria, dehydration, thirst, CNS depression and coma, potential depletion of K+, decreased plasma bicarbonate, dry mucous membranes, breathing difficulties, sweet/fruity breath (acetone), increased acetoacetate (ketone bodies) in the urine. Treating with insulin will stimulate glucose uptake to muscle and adipose tissue from the blood and reduce
hyperglycemia and control ketoacidosis

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17
Q

What steps and enzymes of glycolysis are energy used

A

1 - Hexokinase
3 - Phosphofructokinase-1

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18
Q

What steps and enzymes of glycolysis are energy released

A

6 - Glyceraldehyde 3-phosphate dehydrogenase (NADPH)
7 - Phosphoglycerate kinase (ATP)
10 - Pyruvate kinase (ATP)

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19
Q

Pyruvate kinase deficiency leads to

A

Hemolytic anemia with increased serum 2,3-
BPG levels and reduced ATP production

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20
Q

What are the enzymes in the Luebering-Rapoport Shunt

A

Bisphosphoglycerate mutase
Bisphosphoglycerate phosphotase

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21
Q

What does bisphosphoglycerate mutase do

A

1,3-diphosphoglycerate to 2,3-diphosphoglycerate in LR shunt

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22
Q

What does bisphosphoglycerate phosphatase do

A

2,3-diphosphoglycerate to 3-phosphoglycerate in LR shunt

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23
Q

What is the main enzyme in Methemoglobin Reductase Pathway

A

Cyt b5 reductase

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24
Q

G6PD deficiency leads to

A

hemolytic anemia and prolonged neonatal jaundice due to inability of generating NADPH and pentose sugars. G6PD deficiency provides resistance against malarial infection

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25
Q

Pyruvate kinase deficiency leads to

A

Hereditary hemolytic anemia, increased serum 2,3-BPG levels

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26
Q

In what ways besides glycolysis is dihydroxyacetone phosphate (DHAP) involved

A

AP reduced to glycerol-3-
phosphate (forms glycerol backbone in triglycerides); in gluconeogenesis, it is converted to glyceraldehyde-3-phosphate

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27
Q

What activates pyruvate carboxylase and what does PC do

A

Acetyl Coenzyme A
Catalyzes irreversible
carboxylation of pyruvate to form oxaloacetate. Involved in gluconeogenesis and synthesis of neurotransmitters such as
glutamate.

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28
Q

Deficiency of pyruvate carboxylase leads to

A

an inherited disease that causes lactic acid and other toxic compounds to accumulate in the blood.
The deficiency of this enzyme also causes a decrease in citrate, aspartate, and phosphoenolpyruvate levels as these compounds are formed from oxaloacetate.

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29
Q

What enzyme and coenzyme are associated with E1

A

Pyruvate dehydrogenase (coenzyme: Thiamine pyrophosphate/B1)

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30
Q

What enzyme and coenzyme are associated with E2

A

Dihydrolipoyl transacetylase (coenzymes: Lipoamide, Coenzyme A) (B5)

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31
Q

What enzyme and coenzyme are associated with E3

A

Dihydrolipoyl dehydrogenase (coenzymes: Flavin adenine dinucleotide (FAD) and nicotinamide adenine dinucleotide (NAD+) (B2/B3)

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32
Q

Symptoms of and prognosis of pyruvate dehydrogenase reduced activity

A

Arsenic poisoning
Lactic acidosis, headaches, confusion, convulsions and heart diseases. Arsenic poising can also lead to squamous cell carcinoma

33
Q

PDH reduced activity can be due to

A

Wernicke-Korsakoff (encephalopathy-psychosis syndrome due to thiamine deficiency seen with alcohol abuse)

34
Q

Symptoms of Wernicke-Korsakoff

A

Lactic acidosis, neurological disturbances, paralysis, atrophy of limbs and cardiac failure

35
Q

What is succinate dehydrogenase gene deficiency

A

Homozygous and
heterozygous mutations in the subunits of SD

36
Q

Types of succinate dehydrogenase gene deficiency

A

SDHA: Leigh syndrome (it could also be due to PDH deficiency)
SDHB and SDHD: Paraganglioma and pheochromocytoma
SDHC: Paraganglioma

37
Q

Most common features of succinate dehydrogenase gene deficiency

A

Leigh syndrome, hypertrophic cardiomyopathy, muscle
weakness, cerebral ataxia, optic atrophy

38
Q

What is fumarase gene deficiency

A

Inherited autosomal recessive disease

39
Q

Symptoms of fumarase gene deficiency

A

Affects nervous system
Infants may have an
abnormally small head size, abnormal brain structure, developmental delay, and weak muscle tone, face frontal bossing, depressed nasal bridge, and widely spaced eyes

40
Q

What is succinyl-CoA synthetase gene deficiency

A

Homozygous and heterozygous mutations in the subunits of SUCLA1 and 2

41
Q

What is the importance of succinyl-CoA synthetase

A

Succinyl-coenzyme A
is an intermediate in the TCA cycle and is also a substrate of heme synthesis. Succinyl-coenzyme A and glycine are combined in the first step of heme synthesis to form aminolevulinic acid. In
the Krebs cycle, succinyl-coenzyme A is an intermediate generated by α-ketoglutarate dehydrogenase.

42
Q

Most common symptoms of succinyl-CoA synthetase gene deficiency

A

Ecephalomyopathy, developmental delay,
dystonia, and lactic acidosis

43
Q

Main function of complex I (NADH Oxidoreductase)

A

Transfers electrons from NADH to ubiquinone (CoQ), pumps protons to generate proton gradient

44
Q

Main function of complex II (succinate dehydrogenase)

A

Transfers electrons from FADH2 to ubiquinone (CoQ), does not pump protons (Part of TCA cycle also)

45
Q

Main function of complex III (Ubiquinone-Cytochrome c
Oxidoreductase)

A

Transfers electrons from ubiquinone to cytochrome c, pumps protons

46
Q

Main function of ATP Synthase

A

Uses the proton gradient created by ETC to synthesize ATP from ADP + inorganic phosphate

47
Q

Key inhibitor of complex II (succinate dehydrogenase)

A

Malonate

48
Q

Key inhibitor of complex III (Ubiquinone-Cytochrome c
Oxidoreductase)

A

Antimycin A

49
Q

Key inhibitor of complex IV (Cytochrome c Oxidase)

A

Cyanide

50
Q

Key inhibitor of ATP Synthase

A

Oligomycin

51
Q

Physiological relevance of uncoupling

A

Thermogenesis: UCP1 in brown adipose tissue mediates heat production to maintain body temperature in cold environments

52
Q

Pathological relevance of uncoupling

A

Overuse of uncoupling agents (e.g., DNP, FCCP overdose) can cause hyperthermia, metabolic disturbances, and potentially death

53
Q

What is the MOA of 2,4-Dinitrophenol (DNP)

A

Protonophore that carries protons across the inner
mitochondrial membrane, dissipating the proton gradient and uncoupling oxidative phosphorylation, and heat production

54
Q

What is the MOA of uncoupling Proteins (UCPs)

A

Proteins (e.g., UCP1, UCP2, UCP3) that allow protons to re-enter the mitochondrial matrix without producing ATP, leading to heat generation (thermogenesis).

55
Q

What is carbonyl cyanide-p-
trifluoromethoxyphenylhydrazone (FCCP)

A

An ETC uncoupling agent

56
Q

Medical use for 2,4-Dinitrophenol (DNP)

A

Historically used for weight loss (no longer used due to toxicity, including hyperthermia and fatality)

57
Q

Medical use for uncoupling proteins (UCPs)

A

UCP1 is involved in non-shivering thermogenesis in brown adipose tissue

58
Q

Pyruvate carboxylase cofactor in gluconeogenesis

A

Biotin (B7)

59
Q

How is pyruvate carboxylase activated in gluconeogenesis

A

Acetyl-CoA

60
Q

Process that pyruvate carboxylase is involved in in gluconeogenesis

A

Pyruvate to oxaloacetate (requires malate shuttle into cytosol)

61
Q

Phosphoenolpyruvate carboxykinase (PEPK) cofactor in gluconeogenesis

A

GTP

62
Q

What activates phosphoenolpyruvate carboxykinase (PEPK) in gluconeogenesis

A

Glucagon
Cortisol

63
Q

Process that phosphoenolpyruvate carboxykinase (PEPK) is involved in in gluconeogenesis

A

OAA to phosphoenolpyruvate (PEP) (PEP to fructose-1,6-bisphosphate)

64
Q

Rate limiting step of gluconeogenesis

A

Fructose-1,6-phosphate to fructose-6-phosphate

65
Q

Fructose-1,6-bisphosphatase cofactor in gluconeogenesis

A

ADP

66
Q

What activates/deactivates fructose-1,6-bisphosphatase in gluconeogenesis

A

ATP
AMP and fructose-2,6-phosphate

67
Q

What is nonreversible in gluconeogenesis

A

Glucose-6-Phosphate to glucose

68
Q

What does glucose-6-phosphatase do

A

Glucose-6-Phosphate to glucose

69
Q

Where is glucose-6-phosphatase active

A

Liver

70
Q

Role of fructose 2,6-bisphosphate in gluconeogenesis

A

Controls gluconeogenesis and glycolysis in the liver

71
Q

What produces fructose 2,6-bisphosphate

A

PFK-2

72
Q

What activates/deactivates PFK-2

A

Insulin
Glucagon

73
Q

Glucagon lowers _______ stimulating ________

A

Fructose-2,6-bisphosphate
Gluconeogenesis

74
Q

Insulin increases _______ stimulating ________

A

Fructose-2,6-bisphosphate
Gluconeogenesis

75
Q

Rate limiting step of glycogen synthesis

A

Glycogen synthase

76
Q

What does glycogen synthase do

A

Catalyzes the addition of UDP-glucose to the growing glycogen chain, forming α-1,4-glycosidic bond

77
Q

Rate limiting step of glycogenolysis

A

Glycogen phosphorylase

78
Q

What does glycogen phosphorylase do

A

Catalyzes the cleavage of α-1,4-glycosidic bonds, releasing glucose-1-phosphate (G1P) from
glycogen

79
Q
A