Biochem II Flashcards

1
Q

What is insulin addressing: the BG or energy needs of cells?

A

BG

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2
Q

What is the only source of energy of RBCs?

A

Glycolysis

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3
Q

What is the fuel utilization during the well fed state for the liver?

A

Glucose, AAs

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4
Q

What is the fuel utilization during the well fed state for the muscles?

A

Glucose

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5
Q

What is the fuel utilization during the well fed state for the heart?

A

FAs

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6
Q

What is the fuel utilization during the well fed state for the adipose tissue?

A

Glucose

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7
Q

What is the fuel utilization during the well fed state for the brain?

A

Glucose

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8
Q

What is the fuel utilization during the well fed state for the RBCs?

A

Glucose

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9
Q

What is the fuel utilization during the fasting state for the liver?

A

FAs

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10
Q

What is the fuel utilization during the fasting state for the muscle?

A

FAs, ketones

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11
Q

What is the fuel utilization during the fasting state for the heart?

A

FAs, ketones

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12
Q

What is the fuel utilization during the fasting state for the adipose tissue?

A

FAs

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13
Q

What is the fuel utilization during the fasting state for the Brain?

A

Glucose (ketones in the late fast)

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14
Q

What is the fuel utilization during the fasting state for the RBCs?

A

Glucose

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15
Q

What is the kcal/g for: carbs?

A

4

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16
Q

What is the kcal/g for: fat?

A

9

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17
Q

What is the kcal/g for: EtOH?

A

7

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18
Q

What are the two types of amylase?

A

SAlivary and pancreatic

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19
Q

What are the three different intestinal disaccharidases?

A
  • Lactase
  • Sucrase
  • Maltase
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20
Q

What is the enzyme that is defective in MODY?

A

Glucokinase

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21
Q

What is the transporter that transports glucose from the intestines?

A

SGLT1/2

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22
Q

What is the GLUT that is activated by insulin?

A

GLUT4

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23
Q

What is the GLUT5 activity?

A

Passive uptake of fructose

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24
Q

True or false: fructose is not regulated by insulin, or any other hormone

A

True

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25
Q

Do GLUTs 1-4 utilize passive or active transport?

A

Passive

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26
Q

What is the difference between hexokinase and glucokinase in terms of: location?

A

Hexokinase is in msot tissues, except liver

Glucokinase - only in liver and beta cells of pancrease

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27
Q

What is the difference between hexokinase and glucokinase in terms of: Km?

A

Low for hexokinase (high affinity)

High for glucokinase (low affinity)

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28
Q

What is the difference between hexokinase and glucokinase in terms of: Vmax?

A

Low for hexokinase (low capacity)

High for glucokinase (high capacity)

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29
Q

What is the difference between hexokinase and glucokinase in terms of: inducibility by insulin

A

Hexokinase is not affected by insulin, while glucokinase is not

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30
Q

What is the difference between hexokinase and glucokinase in terms of: Feedback inhibition by G6P

A

Hexokinase is inhibited

Glucokinase is not

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31
Q

What is the main regulated step of glycolysis? What activates and inhibits it?

A
  • Phosphofructokinase is activated via F2,6BP

- Inhibits by ATP, citrate, pH

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32
Q

What is DHAP used for in glycolysis?

A

TAG synthesis

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33
Q

Glycolysis defects produces what diseases?

A

Hemolytic anemias

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34
Q

What is the MOA of arsenate?

A

Inhibits ATP production by glycolysis

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35
Q

What happens to galactose?

A

Converted to glucose

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36
Q

What is the cause of galactosemia? S/sx?

A

Defect in galactose metabolism leads to jaundice, cataracts, and liver damage early in life

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37
Q

What are the two ways in which fructose is degraded?

A
  • Hexokinase side rxn

- Fructokinase in the liver produces F1P, which is degraded by adolase B

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38
Q

What is the cause of benign fructosuria?

A

Defect in fructokinase

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39
Q

What happens with aldolase B deficiency?

A

Hereditary fructose intolerance

  • Jaundice
  • Hypoglycemia
  • Hepatomegaly
40
Q

What is the overall rxn with pyruvate dehydrogenase? Where in the cell is it?

A
  • Produces acetyl-CoA from pyruvate

- mitochondria

41
Q

What are the 5 cofactors that are needed for the Pyruvate dehydrogenase complex?

A

NAD (niacin)
FAD (pantothenate)
B1
B5

42
Q

What activates the Pyruvate dehydrogenase complex? Inhibits?

A
  • Insulin activates

- Repressed by acetyl-CoA

43
Q

What is glycogenin?

A

Enzyme that attaches UDP-glucose to Y residues

44
Q

What is the effect of insulin on liver and muscle glycogen synthase?

A

Activates

45
Q

What inhibits hepatic and muscle glycogen synthase respectively?

A

Hepatic = glucagon and epi

Muscle = epi

46
Q

What activates hepatic and muscle glycogen phosphorylase respectively?

A

Hepatic = glucagon and epi

Muscle = epi

47
Q

What inhibits hepatic and muscle glycogen synthase respectively?

A

Hepatic = insulin

Muscle = insulin

48
Q

What is the enzymatic defect in type I GSD?

A

Glucose-6-phosphate dehydrogenase

49
Q

What is the enzymatic defect in type II GSD?

A

lysosomal glucosidase

50
Q

What is the enzymatic defect in type III GSD?

A

Debranching enzyme

51
Q

What is the enzymatic defect in type IV GSD?

A

Branching enzyme

52
Q

What is the enzymatic defect in type V GSD?

A

Muscle phosphorylase

53
Q

What is the enzymatic defect in type VI GSD?

A

Liver phosphorylase

54
Q

What is the cause of death with Pompe’s disease?

A

LVH

55
Q

What is the general structure of proteoglycans?

A

Long Hyaluronic acid with side branches of Protein cores, with many attached glycosaminoglycans

56
Q

What is the defective enzyme, accumulated products and s/sx of: Hurler’s disease?

A
  • Dermatan sulfate / heparine sulfate
  • alpha-iduronidase
  • Skeletal abnormalities, MR
57
Q

What is the defective enzyme, accumulated products and s/sx of: Hunter syndrome

A

Dermatan sulfate
Heparin sulfate

-Skeletal abnormalities and MR

58
Q

What is the defective enzyme, accumulated products and s/sx of: Sanfiloppo’s disease

A

Heparine sulfate

MR

59
Q

What is the inheritance pattern of Hunter syndrome, hurler syndrome, and sanfilippo>

A
Hunter = XLR
Hurler's = AR
Sanfilippo = AR
60
Q

What are the three molecules that are used as substrates for gluconeogenesis?

A
  • Glycerol P
  • Lactate
  • Glucogenic amino acids
61
Q

What is the rate limiting step of gluconeogenesis?

A

Pyruvate carboxylase

62
Q

Acetyl-CoA regulates what parts of gluconeogenesis?

A

Carboxylase (+) and dehydrogenase (-)

63
Q

Gluconeogenesis depends on what molecule in particular?

A

NAD+

64
Q

Why does EtOH inhibit gluconeogenesis?

A

Lowers NAD+

65
Q

What is the inheritance pattern of G6PD deficiency?

A

AR

66
Q

What type of anemia is produced with G6PD deficiency?

A

Hemolytic

67
Q

What are the three major roles of the PPP?

A
  • liver
  • PMNs = NADPH oxidase
  • RBCs = defend against oxidative stress
68
Q

What is the RBC inclusion for G6PD Deficiency?

A

Heinz bodies

69
Q

What are the essential FAs?

A

Linoleate (9, 12)

70
Q

Where does the synthesis of lipids begin?

A

Export of Acetyl-COa into the cytosol

71
Q

What is the key reaction for lipids in the well fed state?

A

Acetyl-CoA carboxylase

72
Q

What inhibits lipid import into the mitochondria?

A

Malonyl-CoA

73
Q

What is the activating step of FA synthesis?

A

Acyl CoA synthetase

74
Q

Why does EtOH lead to fatty liver changes?

A

Ethanol metabolism produces NADH, which inhibits the citric acid cycle

75
Q

What is the role of albumin in lipid transport?

A

Transports in blood

76
Q

What is the function of ApoA?

A

Lipid uptake into LPs

77
Q

What is the function of ApoB?

A

Structural support (b100 on LDL, B48 on chylomicrons)

78
Q

What is the function of ApoC?

A

For lipid release from LPs (activates LPL)

79
Q

What is the function of ApoE?

A

For removal of LPs via interaction with liver receptor

80
Q

What are the lipoproteins in chylomicrons?

A

b48
C
E

81
Q

What are the lipoproteins in VLDL?

A

B100
C
E

82
Q

What is the general role of LDL?

A

pick up cholesterol, give TAGs

83
Q

What is the protein that extracts lipids from cells for pick up by LPs?

A

ABCA-1

84
Q

What is the protein that is involved in transferring cholesterol between LPs?

A

Cholesteryl ester transfer protein (CTEP)

85
Q
A
86
Q
A
87
Q

LDL oxidation can be prevented by what vitamin?

A

E

88
Q

What is the backbone of HDL particles?

A

Apo A

89
Q

What is familial hyperlipidemia?

A

Elevated LDL and cholesterol 2/2 LDL receptor deficiency

90
Q

What is abetalipoproteinemia?

A

No ApoB100/48, causes lipid accumulation in enterocytes and liver, leading to extremely low serum lipids

91
Q

What is the key step in cholesterol synthesis?

A

HMG-CoA

92
Q

What is the cholesterol intermediate needed for CoQ?

A

Farnesylation

93
Q

What is the shuttle utilized to transport FAs into the mitochondria?

A

Carnitine shuttle

94
Q

What is the key step in FA catabolism? How is it regulated?

A

Carnitine shuttle across the inner mitochondrial membrane

Regulated by malonyl CoA

95
Q

What is the most common defect in FA metabolism?

A

Acyl-coa dehydrogenase

96
Q

Odd chain FAs produce what?

A

propionyl-CoA