lipid metabolism Flashcards

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1
Q

what is the starting product for fatty acid synthesis?

A

citrate (accumulates in the fed state)

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2
Q

where is fatty acid synthesis occurring?

A

cytoplasm

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3
Q

how does citrate move into the cytoplasm?

A

citrate shuttle

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4
Q

how is citrate converted to acetyl-CoA?

A

ATP citrate lyase

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5
Q

how is acetyl coA converted to malonyl CoA? required cofactor?

A

acetyl-CoA carboxylase (RLS), biotin

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6
Q

only ___ fatty acids can be made from malignly coA

A

even chain

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7
Q

where does fatty acid catabolism occur?

A

in the mitochondria

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8
Q

how are fatty acids transported into mitochondria?

A

1) fatty acid coA synthetase converts to acyl-CoA

2) acyl-CoA enters mitochondria via carnitine shuttle

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9
Q

what inhibits the carnitine shuttle?

A

malonyl-CoA (preventing degradation of newly synthesized FAs)

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10
Q

what are the 2 possible fates of B-oxidation products?

A

ketone bodies

TCA cycle

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11
Q

what is the effect carnitine deficiency?

A

decreased carnitine shuttle leads to inability to move LCFA into mitochondria

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12
Q

symptoms of carnitine deficiency (3)

A

weakness
hypotonia
hypokeotoic hypoglycemia

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13
Q

2 substances synthesized from HMG-CoA

A
cholesterol
ketone bodies (acetoacetate)
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14
Q

what is the only energy source of RBCs?

A

glucose

cannot use ketone bodies (lack mitochondria)

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15
Q

ketone bodies can be used for energy because they are converted to

A

2 acetyl-CoA

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16
Q

what is the rate limiting enzyme in ketone body synthesis?

A

HMG CoA synthase

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17
Q

which ketone body is preferred by the brain?

A

b-hydroxybutarate

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18
Q

what gives breath a fruity odor?

A

acetone

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19
Q

where are ketone bodies produced?

A

made in the liver, although the liver lacks the enzymes needed to use them

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20
Q

reaction by HMG-CoA reductase

A

HMG-COA to mevalonate

21
Q

function of LCAT

A

esterifies cholesterol, which allows it to be stored in vesicles (important for transport throughout the body), like HDL

22
Q

pancreatic lipase

A

degrades dietary triglycerides to 2 free FA and monoacylglycerol- allows for absorption into enterocyte

23
Q

lipoprotein lipase location, function

A

endothelium

degrades triglycerides circulating in chylomicrons, VLDL

24
Q

hepatic lipase location, function

A

liver

degrades triglycerides in IDL to form LDL

25
Q

hormone sensitive lipase location, function

A

intracellular

degrades triglycerides in adipocytes

26
Q

CETP

A

mediates transfer of cholesterol to other lipoproteins

27
Q

Apo E

A

mediates remnant uptake into liver

28
Q

ApoA-I

A

activates LCAT

found only on HDL

29
Q

ApoC-II

A

cofactor for lipoprotein lipase

30
Q

apoB-48

A

mediates chylomicron assembly and secretion

31
Q

apoB100

A

binds LDL receptor, mediates release of VLDL from liver, involved in receptor-mediated endocytosis of LDL by cells

32
Q

role of chylomicrons

A

deliver dietary TGs to peripheral tissue

33
Q

role of VLDL

A

deliver TGs from liver to peripheral tissue

34
Q

role of LDL

A

delivers hepatic cholesterol to peripheral tissues

35
Q

why does LDL contain mostly cholesterol?

A

VLDL was acted on by lipoprotein lipase to deliver triglycerides to tissue, leaving cholesterol

36
Q

role of HDL

A

carries cholesterol from the periphery to the liver

37
Q

type I hyperchylomicronemia increases (3)

A

chylomicrons, triglycerides, cholesterol

38
Q

2 causes of type I hyperchylomicronemia

A

AR defect of lipoprotein lipase or Apo C-II

39
Q

symptoms of type I hyperchylomicronemia

A

pancreatitis, xanthomas

*no increased risk for atherosclerosis

40
Q

type IIa familial hypercholesterolemia increases (2)

A

LDL, cholesterol

41
Q

cause of type IIa familial hypercholesterolemia

A

AD decreased or absent LDL receptors

42
Q

symptoms of type IIa hypercholesterolemia

A

atherosclerosis, tendinous xanthomas, corneal arcus

43
Q

type III dysbetalipoproteinemia is caused by:

A

defects in ApoE, leads to increased chylomicron remnants in the circulation

44
Q

type IV hypertriglyceridemia increases (2)

A

VLDL, triglycerides

45
Q

type IV hypertriglyceridemia is caused by:

A

AD hepatic overproduction of VLDL, absent ApoA-V

46
Q

symptoms of type IV hypertriglycermidemia

A

pancreatitis

47
Q

what causes abetalipoproteinemia?

A

AR lack of B48 and B100 due to mutation in MTP gene leads to decreased chylomicron and VLDL synthesis and secretion, fat cannot be absorbed and instead, accumulates in enterocytes

48
Q

symptoms of abetalipoproteinemia (4)

A

steatorrhea, acanthocytosis, ataxia, night blindness