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M2M unit 1 > Prion Vignette and Alzheimers > Flashcards

Prion Vignette and Alzheimers Flashcards

1
Q

which is soluble in mild detergent PrPc or PrPsc?

A

PrPc

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2
Q

how are AB40 and AB42 formed

A

cleavage by beta secretase followed by gamma secretase

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3
Q

Favored secretase pathway

A

alpha secretase followed by gamma secretase

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4
Q

where do most AD cases come from?

A

95% are 65+

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5
Q

Prion Strains

A

characteristic features that are variable: incubation time in host, clinical signs, distribution of protease resistance PrP in brain. these are stable on serial propagation and posses certian bio-chemcial properties

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6
Q

Role of APP in AD

A

APP is on chromosome 21 mutation in APP area lead to early onset AD trisomy 21 with 3 copies of APP leads to earlier development of AD

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7
Q

Where does gamma secretase makes its cleave?

A

Transmembrane region 700-723

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8
Q

what is early age onselt

A

5-6% of AD

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9
Q

Apolipoprotein E 4

A

key role in amyloid processing and clearance if you have ApE4, you have more amyloid plaques and AD increases risk by 2 fold, decrease time of onset

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10
Q

Inherited Prion

A

10% of cases familial strains that lead to early onset CJD, Gerstmann-Straussler Syndrome, Fatal familial insomnia

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11
Q

Apolipoprotein E 4

A

key role in amyloid processing and clearance if you have ApE4, you have more amyloid plaques and AD increases risk by 2 fold, decrease time of onset

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12
Q

Favored secretase pathway

A

alpha secretase followed by gamma secretase

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13
Q

Inherited Prion

A

10% of cases familial strains that lead to early onset CJD, Gerstmann-Straussler Syndrome, Fatal familial insomnia

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14
Q

Presenilin 1

A

on Chromosome 14 missense mutation that alters single amino acid may be the gamma secretase itself increased production of Beta amyloid protein 42

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15
Q

which is soluble in mild detergent PrPc or PrPsc?

A

PrPc

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16
Q

Tau

A

protein that leads to fibrillary tangles inside the cell

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17
Q

how do we increas AB42 clerance?

A

choleserol or statins

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18
Q

Structural differences in PrPc and PrPsc?

A

PrPc is alpha helical PrPsc is beta sheets that are hydrophobic and clump

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19
Q

Role of APP in AD

A

APP is on chromosome 21 mutation in APP area lead to early onset AD trisomy 21 with 3 copies of APP leads to earlier development of AD

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20
Q

Infectious

A
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21
Q

Decrease inflammatory response of AD

A

NSAIDS, steriods

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22
Q

what is early age onselt

A

5-6% of AD

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23
Q

where do AB42 come from?

A

beta secretase cleavage followed by gamma secretase action on APP

24
Q

PrPc vs PrPsc

A

PrPc is the healthy prion protein in all animals PrPsc is the unhealthy prion protein only in diseased animals PrPc is soluble in mild detergent, sensitive to digestion, alpha helix, unstructured N terminis, little beta sheets, globulr 3°, 4° mono oligo-meric PrPsc: not soluble, digestion resistant core, made mostly of beta sheets and are hydrophobic and clump in lesions, 3° structure unknown; 4° is beta sheet linked aggregates. both have identical covalent structure

25
Q

How do we decrease AB42 toxicity

A

decrease oligimerization, protect mitochondira

26
Q

what is sensitive to digestion? PrPc or PrPsc?

A

PrPc

27
Q

where do AB42 come from?

A

beta secretase cleavage followed by gamma secretase action on APP

28
Q

Tau

A

protein that leads to fibrillary tangles inside the cell

29
Q

where do most AD cases come from?

A

95% are 65+

30
Q

Structural differences in PrPc and PrPsc?

A

PrPc is alpha helical PrPsc is beta sheets that are hydrophobic and clump

31
Q

Presenilin 2 mutaion

A

on chromosome 1 increase BA42

32
Q

what is sensitive to digestion? PrPc or PrPsc?

A

PrPc

33
Q

relationship between BSE and vCJD

A

BSE is mad cow disease and vCJD is strain of prion disease that originated in cow and through dietary ingestion lead to vCJD in humans

34
Q

Sporadic Prion

A

90% of cases due to mis-folding that infects rest of prions CJD

35
Q

Beta-Amyloid 42 plaque

A

incorrectly cleaved protein that forms from A-42 insoluble plaques formed extracellularly

36
Q

how is APP cleaved?

A

alpha secretase or Beta secretase transmembrane cleavage by gamma secretase

37
Q

Prion Strains

A

characteristic features that are variable: incubation time in host, clinical signs, distribution of protease resistance PrP in brain. these are stable on serial propagation and posses certian bio-chemcial properties

38
Q

decrease damage to synapses/neurons

A

memantine

39
Q

what decreases AB

A

Iclanders have a mutation near beta cleavage site 40% less AB and decreased risk of AD

40
Q

Beta secretase knockout

A

mice are okay, so we are looking at selective beta secretase inhibitors

41
Q

problem with alzheimers drugs?

A

they target too low down on the activation pathway. We need to target it before the AB42 starts to accumulate in the brain and symptoms begin developing

42
Q

how is APP cleaved?

A

alpha secretase or Beta secretase transmembrane cleavage by gamma secretase

43
Q

Infectious

A
44
Q

What types of prion disease are there?

A

Sporadic, inherited, infectious

45
Q

how do we need to decrease AB42 production

A

modulate secretase activity

46
Q

PrPc vs PrPsc

A

PrPc is the healthy prion protein in all animals PrPsc is the unhealthy prion protein only in diseased animals PrPc is soluble in mild detergent, sensitive to digestion, alpha helix, unstructured N terminis, little beta sheets, globulr 3°, 4° mono oligo-meric PrPsc: not soluble, digestion resistant core, made mostly of beta sheets and are hydrophobic and clump in lesions, 3° structure unknown; 4° is beta sheet linked aggregates. both have identical covalent structure

47
Q

Presenilin 2 mutaion

A

on chromosome 1 increase BA42

48
Q

What types of prion disease are there?

A

Sporadic, inherited, infectious

49
Q

Beta-Amyloid 42 plaque

A

incorrectly cleaved protein that forms from A-42 insoluble plaques formed extracellularly

50
Q

Presenilin 1

A

on Chromosome 14 missense mutation that alters single amino acid may be the gamma secretase itself increased production of Beta amyloid protein 42

51
Q

APP

A

B-amyloid precursor protein large ubiqutiously procued protein in mammalian cells transmembrane protein that is processed by several enzymatic cleavages coded on chromosome 21

52
Q

APP

A

B-amyloid precursor protein large ubiqutiously procued protein in mammalian cells transmembrane protein that is processed by several enzymatic cleavages coded on chromosome 21

53
Q

relationship between BSE and vCJD

A

BSE is mad cow disease and vCJD is strain of prion disease that originated in cow and through dietary ingestion lead to vCJD in humans

54
Q

how are AB40 and AB42 formed

A

cleavage by beta secretase followed by gamma secretase

55
Q

Gamma secretase knockout

A

knock out model is lethal seek to modulate activity

56
Q

Sporadic Prion

A

90% of cases due to mis-folding that infects rest of prions CJD

57
Q

what decreases AB

A

Iclanders have a mutation near beta cleavage site 40% less AB and decreased risk of AD

M2M unit 1 (6 decks)

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