Pathology with protein folding Flashcards Preview

Jonathan's Neuroscience > Pathology with protein folding > Flashcards

Flashcards in Pathology with protein folding Deck (17):
1

Is Alzheimer's a part of normal ageing process?

No, because there is abnormal protein folding

2

Why can't abnormally folded proteins be effectively cleared?

these proteins develop postnatally, and the body is not adapted to clear these protein complexes

3

What are the genes involved in AD?

APP
Apolipoprotein E
PS1
PS2

4

Which gene is related to sporadic late onset AD?

Apo E

5

Where do AD normally start its effects

medial temporal and frontal lobes

6

What are the two principle pathologies related to AD?

NFT - neurofibriin tangle, associated with Tau
APC - formation of amyloid plaque

7

How are amyloid plaques formed?

APP, a transmembrane protein is cleaved by enzyme to form Ab monomers. The aggregation of monomers forms insoluble oliogomers, and eventually becomes a plaque

8

What is the enzyme that cleaves the APP?

y-secretase

9

Why can't we target y-secretase for AD?

the enzyme cuts almost all type 1 transmembrane proteins, not just APP, so inhibiting the enzyme will inhibit many normal processes

10

T/F amyloid plaque doesn't do any damage to the brain

True, it's the Ab monomers that disrupts synaptic transmissions

The plaque is just a biomarker of AD

11

What kind of disease is Creutzfeldt-Jakob Disease?

prion disease

12

What are the symptoms of Kuru disease?

tremors and pathologic bursts of laughter

13

How is Kuru disease transmitted?

cannibalism

14

What is BSE disease also known as? Which disease is it similar to?

mad cow disease
it's similar to kuru

15

How does mutation occur in BSE

At the PrP gene, the methionine at 129 (instead of valine) determines the risk of developing mutation. Mutation leads to expansion, which ultimately creates an abnormal, autosomal dominant pattern

16

What is CJD commonly known as?

the human form of mad cow disease

17

in Parkinsons', how does a fibril form?

DA + a-synuclein + metal rich environment = fibril formation