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Biochem > Lipid Synthesis > Flashcards

Lipid Synthesis Flashcards

1
Q

Main site of fatty acid synthesis from excess glucose

A

Liver

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2
Q

Enzyme that catalyzes:
Citrate + ATP → oxaloacetate + acetyl CoA + ADP

A

Citrate lyase

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3
Q

Source of cytosolic acetyl CoA

A

Citrate

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4
Q

Enzyme that catalyzes:
Oxaloacetate + NADH → Malate + NAD+

A

Cytosolic malate dehydrogenase
(reverse rxn done in mitochondria TCA cycle)

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5
Q

Enzyme that catalyzes:
malate + NADP+ → pyruvate + CO2 + NADPH

A

Malic enzyme

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6
Q

Subcellular location of fatty acid synthesis

A

Cytosol

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7
Q

Enzyme that catalyzes:
Acetyl CoA –> malonyl CoA

A

Acetyl CoA carboxylase

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8
Q

Requirements of acetyl CoA carboxylase (3)

A

CO2, ATP, biotin

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9
Q

Inhibition of acetyl CoA carboxylase (2)

A

Palmitoyl CoA (feedback inhibition; end product of fatty acid synthase)
AMP-dependent protein kinase inactivates (low energy/epinephrine)

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10
Q

Activation of acetyl CoA carboxylase (2)

A

Citrate (feed forward activation)
Insulin (activates a phosphatase)

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11
Q

Enzyme that catalyzes:
Malonyl CoA → → → Palmitoyl CoA

A

Fatty acid synthase

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12
Q

Enzyme with 7 separate enzymatic activities that produces palmitoyl CoA from malonyl CoA

A

Fatty acid synthase

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13
Q

Source of all carbons except the first 2 in fatty acid synthase

A

Malonyl Coa

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14
Q

Malonyl CoA loses it transient carboxyl as this

A

CO2

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15
Q

End product of fatty acid synthesis

A

Palmitate (palmitoyl CoA)

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16
Q

Elongation of fatty acids beyond C16 occurs in this subcellular location

A

ER

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17
Q

Elongation of fatty acids beyond C16 requires these

A

Malonyl CoA and NADPH

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18
Q

Desaturation of fatty acids occurs in this subcellular location

A

ER

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19
Q

Humans can’t add double bonds beyond this carbon in fatty acids

A

C9

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20
Q

Precursor fatty acid for arachidonic acid

A

Linoleic acid

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21
Q

Arachidonic acid is metabolized to these (3)

A

Prostaglandins
Thromboxanes
Leukotrienes

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22
Q

Do cis or trans fatty acids increase the incidence of coronary heart disease?

A

Trans

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23
Q

Type of fatty acids that decrease risks of heart disease

A

Common poly unsaturated fatty acids (cis double bonds) = PUFA’s

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24
Q

Fish oil is rich in these two omega 3 fatty acids

A

EPA and DHA

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25
Q

EPA and DHA omega 3 fatty acids are made from this by elongation and desaturation

A

Linolenic acid

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26
Q

Components of fish oil that are cardioprotective

A

EPA and DHA omega 3 fatty acids

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27
Q

Triglycerides are synthesized from these

A

Glycerol-3-phosphate and fatty acyl CoA derivative

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28
Q

Glycerol-3-phosphate and fatty acyl CoA derivatives form this

A

Phosphatidic acid

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29
Q

Tissue where alpha glycerol phosphate may be derived from glucose, glycerol, or gluconeogenesis

A

Liver

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30
Q

Tissue where alpha glycerol phosphate may be derived from glucose but NOT from glycerol since it lacks glycerol kinase

A

Adipose

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31
Q

Deficiencies of this compound are rare since it is abundant in food except in cases ot total parenteral nutrition

A

Choline

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32
Q

Deficiencies in choline lead to this

A

Fatty liver
(inability to form phospholipids for VLDL)

33
Q

Surfactant in lung that allows the terminal air sacs (alveoli) to inflate easily

A

Dipalmitoyl-phosphatidyl choline (lecithin)

34
Q

This ratio in amniotic fluid is an indicator of lung maturity of the fetus

A

Lecithin to sphingomyelin ratio

35
Q

Lecithin levels rise rapidly after this many weeks gestation

A

35

36
Q

Phospholipase present in many tissues and pancreatic juice

A

Phospholipase A2

37
Q

Phospholipase that acts on phosphatidyl-inositol, releasing arachidonic acid

A

Phospholipase A2

38
Q

Phospholipase A2 acts on phosphatidyl-inositol to release this

A

Arachidonic acid

39
Q

Precursor of prostaglandins

A

Arachidonic acid

40
Q

Proenzyme phospholipase A2 is activated by this and requires this for activity

A

Activated by trypsin
Requires bile salts

41
Q

Phospholipase inhibited by glucocorticoids

A

Phospholipase A2

42
Q

Phospholipase A2 is inhibited by

A

Glucocorticoids

43
Q

Phospholipase found in liver lysosomes

A

Phospholipase C

44
Q

Phospholipase that is activated by the PIP2 system and thus plays a role in producing second messengers

A

Membrane bound phospholipase C

45
Q

Enzyme that catalyzes the first step in the synthesis of prostaglandins and thromboxanes from arachidonic acid

A

Cyclo-oxygenase

46
Q

Arachidonic acid is a precursor that can form these 2 types of molecules (catalyzed by cyclo-oxygenase)

A

Prostaglandins
Thromboxanes

47
Q

Cyclo-oxygenase is inhibited by this

A

Aspirin

48
Q

Aspirin is an irreversible covalent inhibitor of this enzyme

A

Cyclo-oxygenase

49
Q

Product of cyclo-oxygenase that facilitates platelet aggregation and its formation is blocked by aspirin

A

Thromoxane TxA2

50
Q

Product of cyclo-oxygenase that inhibits platelet aggregation

A

Prostacyclin I2

51
Q

Rare but serious condition that causes systemic issues, in particular swelling of the liver and brain
Mechanism involves mitochondrial injury resulting in a dysfunction in oxidative phosphorylation

A

Reye’s syndrome

52
Q

Is hypoglycemia or hyperglycemia seen in Reye’s Syndrome?

A

Hypoglycemia

53
Q

In sphingolipid synthesis, phosphocholine and other phospholipid head groups are activated generally as this

A

CDP derivatives

54
Q

Molecule that is a sphingosine and a fatty acid

A

Ceramide

55
Q

Blood group determinants are based on this

A

Sphingolipids; differ in sugars present on glycosphingolipids

56
Q

Type of diseases resulting from defective lysosomal enzymes used in degradation of sphingolipids
Most cause intellectual disability

A

Sphingolipidoses

57
Q

Sphingolipidosis that involves accumulation of gangliosides Gm2 due to deficiency of Hexosaminidase A

A

Tay-Sachs disease

58
Q

Sphingolipidosis due to deficiency of hexosaminidase A

A

Tay-Sachs disease

59
Q

Sphingolipidosis that involves accumulation of gangliosides Gm2

A

Tay-Sachs disease

60
Q

Cherry-red macula is seen in these 2 sphingolipidoses

A

Tay-Sachs disease
Niemann-Pick disease

61
Q

Sphingolipidosis that is an accumulation of gluco-cerebrosides due to deficiency of Beta-glucosidase

A

Gaucher disease

62
Q

Sphingolipidosis caused by deficiency of beta-glucosidase

A

Gaucher disease

63
Q

Sphingolipidosis that involves accumulation of gluco-cerebrosides

A

Gaucher disease

64
Q

Sphingolipidosis that involves “crumpled tissue paper” appearance of the cytoplasm caused by enlarged, elongated lysosomes

A

Gaucher disease

65
Q

“Crumpled tissue paper” appearance of the cytoplasm of Gaucher cells is caused by enlarged, elongated lysosomes filled with this

A

Glucocerebrosides

66
Q

Sphingolipidosis that involves an accumulation of Gm2 and globosides due to deficiency of beta-hexosaminidase A and B

A

Sandhoff disease

67
Q

Condition that involves the same neurological symptoms as Tay-Sachs but with visceral involvement as well

A

Sandhoff disease

68
Q

Only Sphingolipidosis that is X-liniked rather than autosomal recessive

A

Fabry disease

69
Q

Sphingolipidosis that involves accumulation of globosides due to deficiency of alpha-glucosidase

A

Fabry disease

70
Q

Sphingolipidosis that involves accumulation of globosides

A

Fabry disease

71
Q

Sphingolipidosis that is due to deficiency of alpha-glucosidase

A

Fabry disease

72
Q

Sphingolipidosis that invovles a reddish-purple skin rash

A

Fabry disease

73
Q

Sphingolipidosis that involves accumulation of sphingomyelin due to deficiency of sphingomyelinase

A

Niemann-Pick disease

74
Q

Sphingolipidosis that involves accumulation of sphingomyelin

A

Niemann-Pick disease

75
Q

Sphingolipidosis that is due to deficiency of sphingomyelinase

A

Niemann-Pick disease

76
Q

Sphingolipidoses that involve hepatosplenomegaly

A

Gaucher disease and Niemann-Pick disease

77
Q

Disease that involves foamy-appearing cells

A

Niemann-Pick disease

78
Q

Foamy-appearing cells in Niemann-Pick disease contain this

A

Sphingomyelin

Biochem (21 decks)

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