Lipids

Question 1

Deficiency of Apo B-100, Apo B-48

Answer 1

abetalipoproteinemia

Question 2

Result of Apo B-100, Apo B-48 deficiency

Answer 2

enterocytes cannot release lipids into lymphatics
decreased chylomicrons
decreased VLDL synthesis

Question 3

Gene mutation in abetalipoproteinemia

Answer 3

MTP

microsomal transfer protein

Question 4

Clinical features of abetalipoproteinemia

Answer 4

failure to thrive
steatorrhea
ADEK malabsorption
night blindness (vit A)
ataxia (vit A)

Question 5

RBC pathology of abetalipoproteinemia

Answer 5

acanthocytosis

misshapen, spiky RBC membrain due to lipid problem

Question 6

Treatment of abetalipoproteinemia

Answer 6

Vit E

Question 7

Deficiency of lipoprotein lipase (could be due to Apo C-II defect)

Answer 7

Type-I hyperchylomicronemia (autosomal recessive)

Question 8

Function of lipoprotein lipase

Answer 8

hydrolyzes TAGs found on chylomicrons and VLDL

as a result there is increased chylomicrons and cholesterol in blood

Question 9

Product of lipoprotein lipase

Answer 9

2 FFA’s, 1 monoacylglycerol

Question 10

Presentation of type-I hyperchylomicronemia

Answer 10

pancreatitis (increased TAGs)
hepatosplenomegaly
pruritic xanthoma
NO increased risk of atherosclerosis

Question 11

Absent or decreased LDL receptors

Answer 11

type IIA familial hypercholesterolemia

no LDL receptor = no cholesterol taken in by cells

Question 12

tendinous xanthomas
corneal arcus
accelerated atherosclerosis (MI risk)

Answer 12

type IIA familial hypercholesterolemia

increased MI risk in younger, otherwise healthy individuals

Question 13

Overproduction of VLDL in liver: what disease

Answer 13

Type IV hypertriglyceridemia

assoc with increased risk of pancreatitis

Question 14

Activates LCAT

Answer 14

Apo A-I

Question 15

Mediates chylomicron secretion

Answer 15

Apo B-48

Question 16

Mediates VLDL secretion

Answer 16

Apo B-100

Question 17

Cofactor for lipoprotein lipase

Answer 17

Apo C-II

Question 18

Mediates uptake of remnant particles

Answer 18

Apo E

Question 19

binds to LDL receptor

Answer 19

Apo B-100

Question 20

RLE of fatty acid synthesis

Answer 20

acetyl CoA carboxylase

Question 21

RLE of fatty acid B-oxidation

Answer 21

carnitine acyltransferase-1/palmitoyl-1

Question 22

RLE of ketone body synthesis

Answer 22

HMG-CoA synthase

Question 23

RLE of cholesterol synthesis

Answer 23

HMG-CoA reductase

Question 24

inhibits RLE of cholesterol synthesis

Answer 24

statins