Lipids IV

Question 1

what does degradation of glycolipids require

Answer 1

sequential action of different glycosidases with high substrate specificty

Question 2

defect in tay sachs

Answer 2

beta hexosamidase A

Question 3

what accuulates in tay sachs

Answer 3

ganglioside

Question 4

symptoms of tay sachs

Answer 4

intellectual disability
blindness
cherry macula
death before age 3

Question 5

defect in gaucher

Answer 5

beta glucosidase

Question 6

what accumulates in gaucher disease

Answer 6

glucocerebroside

Question 7

symptom of gaucher disease

Answer 7

liver and spleen enlargement

erosion o flong bones

Question 8

defect in fabry disease

Answer 8

alpha-galactosidase

Question 9

what accumulates in fabry disease

Answer 9

ceramide rihexoside

Question 10

symptom of fabry disease

Answer 10

skin rash

kidney failure

Question 11

defect in neimann pick disease

Answer 11

spingomyelinase

Question 12

what accumualtes in neimann pick diseas

Answer 12

sphingomylen

Question 13

presentation of neimann pick disease

Answer 13

liver and splenn enlargement

intelluctal disability

Question 14

defect in sandhoff disaes

Answer 14

beta hexosamindase A and B

Question 15

what accumulates in sandhoff disease

Answer 15

ganglioside

globosides

Question 16

what is symptoms of sandhoff disease

Answer 16

like tay sachs but progresses more rapidly

Question 17

defect in metachromatic leukodystrophy

Answer 17

arysulfatase

Question 18

what accumulates in metachromatic leukodystrophy

Answer 18

sulfatide

Question 19

what is the presentation of metachromatic leukodystrophy

Answer 19

intellectual disablity

Question 20

when does synthesisi of cholesterol happen

Answer 20

well fed state

Question 21

what is the rate limiting step of synthesis of cholesterol

Answer 21

HMG-CoA reductase reaction

Question 22

what is farnesyl-PP important for

Answer 22

making dolichol-P
prenylation of proteins
precursoe for coenzyme Q

Question 23

waht is dolichol-P used for

Answer 23

N-linked glycosylation of proteins

Question 24

what is prenylation of proetins used for

Answer 24

post translation addition of a lipid to membrane anchored proteins

Question 25

when is HMG CoA active

Answer 25

cellular energy is high
blood glucose is high
intracellular cholesterol conc is low

Question 26

activity of HMG-CoA can be reduced by what drug

Answer 26

statin

Question 27

what type of properties does cholesterol have

Answer 27

amphipolar

Question 28

characteristic of cholesteryl ester

Answer 28

compleletly hydrophobic

Question 29

where are cholesteryl esters found

Answer 29

hydrophobic center of lipoproteins, cytoplasmic fat droplets

Question 30

characteris of bile acids

Answer 30

water solbule