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M2M unit 1 > Prion Vignette and Alzheimers > Flashcards

Prion Vignette and Alzheimers Flashcards

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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?

Study These Flashcards
A

beta secretase cleavage followed by gamma secretase action on APP

24
Q

PrPc vs PrPsc

Study These Flashcards
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
How do we decrease AB42 toxicity
decrease oligimerization, protect mitochondira
26
what is sensitive to digestion? PrPc or PrPsc?
PrPc
27
where do AB42 come from?
beta secretase cleavage followed by gamma secretase action on APP
28
Tau
protein that leads to fibrillary tangles inside the cell
29
where do most AD cases come from?
95% are 65+
30
Structural differences in PrPc and PrPsc?
PrPc is alpha helical PrPsc is beta sheets that are hydrophobic and clump
31
Presenilin 2 mutaion
on chromosome 1 increase BA42
32
what is sensitive to digestion? PrPc or PrPsc?
PrPc
33
relationship between BSE and vCJD
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
Sporadic Prion
90% of cases due to mis-folding that infects rest of prions CJD
35
Beta-Amyloid 42 plaque
incorrectly cleaved protein that forms from A-42 insoluble plaques formed extracellularly
36
how is APP cleaved?
alpha secretase or Beta secretase transmembrane cleavage by gamma secretase
37
Prion Strains
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
decrease damage to synapses/neurons
memantine
39
what decreases AB
Iclanders have a mutation near beta cleavage site 40% less AB and decreased risk of AD
40
Beta secretase knockout
mice are okay, so we are looking at selective beta secretase inhibitors
41
problem with alzheimers drugs?
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
how is APP cleaved?
alpha secretase or Beta secretase transmembrane cleavage by gamma secretase
43
Infectious
44
What types of prion disease are there?
Sporadic, inherited, infectious
45
how do we need to decrease AB42 production
modulate secretase activity
46
PrPc vs PrPsc
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
Presenilin 2 mutaion
on chromosome 1 increase BA42
48
What types of prion disease are there?
Sporadic, inherited, infectious
49
Beta-Amyloid 42 plaque
incorrectly cleaved protein that forms from A-42 insoluble plaques formed extracellularly
50
Presenilin 1
on Chromosome 14 missense mutation that alters single amino acid may be the gamma secretase itself increased production of Beta amyloid protein 42
51
APP
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
APP
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
relationship between BSE and vCJD
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
how are AB40 and AB42 formed
cleavage by beta secretase followed by gamma secretase
55
Gamma secretase knockout
knock out model is lethal seek to modulate activity
56
Sporadic Prion
90% of cases due to mis-folding that infects rest of prions CJD
57
what decreases AB
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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