Lipids IV Flashcards Preview

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Flashcards in Lipids IV Deck (30):
1

what does degradation of glycolipids require

sequential action of different glycosidases with high substrate specificty

2

defect in tay sachs

beta hexosamidase A

3

what accuulates in tay sachs

ganglioside

4

symptoms of tay sachs

intellectual disability
blindness
cherry macula
death before age 3

5

defect in gaucher

beta glucosidase

6

what accumulates in gaucher disease

glucocerebroside

7

symptom of gaucher disease

liver and spleen enlargement
erosion o flong bones

8

defect in fabry disease

alpha-galactosidase

9

what accumulates in fabry disease

ceramide rihexoside

10

symptom of fabry disease

skin rash
kidney failure

11

defect in neimann pick disease

spingomyelinase

12

what accumualtes in neimann pick diseas

sphingomylen

13

presentation of neimann pick disease

liver and splenn enlargement
intelluctal disability

14

defect in sandhoff disaes

beta hexosamindase A and B

15

what accumulates in sandhoff disease

ganglioside
globosides

16

what is symptoms of sandhoff disease

like tay sachs but progresses more rapidly

17

defect in metachromatic leukodystrophy

arysulfatase

18

what accumulates in metachromatic leukodystrophy

sulfatide

19

what is the presentation of metachromatic leukodystrophy

intellectual disablity

20

when does synthesisi of cholesterol happen

well fed state

21

what is the rate limiting step of synthesis of cholesterol

HMG-CoA reductase reaction

22

what is farnesyl-PP important for

making dolichol-P
prenylation of proteins
precursoe for coenzyme Q

23

waht is dolichol-P used for

N-linked glycosylation of proteins

24

what is prenylation of proetins used for

post translation addition of a lipid to membrane anchored proteins

25

when is HMG CoA active

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

26

activity of HMG-CoA can be reduced by what drug

statin

27

what type of properties does cholesterol have

amphipolar

28

characteristic of cholesteryl ester

compleletly hydrophobic

29

where are cholesteryl esters found

hydrophobic center of lipoproteins, cytoplasmic fat droplets

30

characteris of bile acids

water solbule