Lipids Flashcards

1
Q

What do intestinal enterocytes make from broken down fats

A

Chylomicrons (90% TG)

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2
Q

Where do chylomicrons go once made

A

Lymphatic system –> thoracic duct–> blood

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3
Q

What is required for transport of chylomicrons out of the small intestine

A

Apo B48

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4
Q

What happens if not enough Apo B48

A

Abetalipoproteinemia

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5
Q

What do cells use to draw free FFA’s from circulating chylomicrons

A

Lipoprotein Lipase

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6
Q

What does liver do with chylomicron remnat

A

Makes VLDL

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7
Q

What does Apo B 100 do

A

Helps secrete VLDL that was made in the liver

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8
Q

What happens if not enough Apo B 100

A

VLDL cannot leave liver

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9
Q

What is VLDL transformed to as FFA’s are taken up by cells

A

IDL

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10
Q

What is IDL degraded to and by what?

A

LDL, by hepatic triglyceride lipase - made in liver

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11
Q

What is LDL rich in

A

Cholesterol - lost FFA’s

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12
Q

What happens if LDL oxidized

A

Atheroma

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13
Q

How do cells absorb LDL

A

By endocytosis, using clathrin coated pit, LDL receptor

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14
Q

What can cells do if they have too much cholesterol

A

Deposit it in circulating HDL from liver

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15
Q

What is LCAT

A

HDL uptakes cholesterol from cells this way

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16
Q

What can HDL deposit cholesterol on? By what enzyme?

A

VLDL or LDL via CETP

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17
Q

What is SRB1

A

Scavenger receptor - for hepatocyte to pull cholesterol off HDL

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18
Q

Apo B48

A

Chylomicron secretion from enterocyte to lymphatic system

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19
Q

Apo B100

A

Found on VLDL, IDL, and LDL that have left the liver

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20
Q

Apo E

A

Mediate extra remnant uptake

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21
Q

Apo A-1

A

Activates LCAT, found on HDL

22
Q

Apo C-II

A

Cofactor for LPL

23
Q

What is deficient in Abetalipoproteinemia

A

AR, deficiency in Apo-B48 and ApoB-10

Mutation in MTP gene

24
Q

Presentation of abetalipoproteinemia

A

Presents in first few months of life with FTT, steatorrhea, impaired transport of fat soluble vitamins (ADEK), acanthocytosis (misshapen RBC membrane bc of bad lipids), ataxia, night blindness

25
Q

Treatment of abetalipoproteinemia

A

Vitamin E

26
Q

What is defective in Type 1 Hyperchylomicronemia

A

AR
Deficiency of LPL
Or, Defective Apolipoprotein CII (LPL cofactor)

27
Q

Presentation of Type 1 Hyperchylomicronemia

A

Pancreatitis, HSM, xanthomas

No incr risk of atherosclerosis

28
Q

What is elevated in the blood in Type 1 Hyperchylomicronemia

A

Chylomicrons, TG, Cholesterol

29
Q

What is defective in Type IIa Familial hypercholesterolemia

A

AD

Absent/decreased LDL receptor - needed for uptake of circulating LDL

30
Q

Presentation Type IIa Familial hypercholesterolemia

A

Tendinous xanthomas
Corneal arcus
Accelerated Atherosclerosis

31
Q

What is defective in Type IV hypertriglyceridemia

A

AD

Overproduction of VLDL in liver

32
Q

What is elevated in blood in Type IV hypertriglyceridemia

A

VLDL and TG

33
Q

Presentation of type IV hypertriglyceridemia

A

Pancreatitis

34
Q

What is increased in blood in type IIa familial hypercholesterolemia?

A

LDL and cholesterol

35
Q

What is the substrate for making cholesterol in the body?

A

Acetyl CoA

36
Q

Rate limiting enzyme of cholesterol synthesis

A

HMG-CoA reductase

37
Q

What is the main precursor in FA synthesis

A

Acetyl CoA

38
Q

Where does FA synthesis take place

A

Cytoplasm of hepatocytes

39
Q

Rate limiting enzyme of FA synthesis

A

Acetyl-CoA carboxylase

40
Q

Where does FA degradation take place

A

Mitochondria

41
Q

What is rate-limiting enzyme of FA degradation (beta-oxidation)

A

Carnitine acyltransferase-1

42
Q

What happens if deficient in CAT-1?

A

Can’t get long chain FA into mt –> accum in cytoplasm. And can’t be broken down.

43
Q

Activates LCAT

A

Apo A-1

44
Q

LCAT

A

Catalyzes esterification of cholesterol

45
Q

Mediates chylomicron secretion

A

Apo B-48

46
Q

Mediates VLDL secretion

A

Apo B-100

47
Q

Apoprotein that Binds LDL receptor

A

Apo B-100

48
Q

Cofactor for lipoprotein lipase

A

Apo C-II

49
Q

Mediates uptake of remnant particles

A

Apo E

50
Q

Rate limiting enzyme for ketone body synthesis

A

HMG-CoA synthase