Prion Disease and ALS Flashcards

1
Q

What prion disease and in what animal was first to be identified?

A

Scrapie in sheep.

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2
Q

What is the precursor protein of prions?

A

Prion protein, a cell-surface glycoprotein.

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3
Q

What is the function of prion protein, a cell-surface glycoprotein?

A

Not certain, is copper binding

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4
Q

What is the major secondary structure of prion protein?

A

Alpha-helical

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5
Q

What is the structure of prion protein?

A

Alpha helical globular structure and random-coil hydrophobic structure with some glutamine repeats at the N-terminus.

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6
Q

What are the two states that prion protein can be in and which is susceptible to protease digestion?

A

Scrapie and cellular (susceptible)

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7
Q

What part of the prion protein (PrP) is necessary for disease?

A

Anchor

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8
Q

Is hamster-mouse transmission of prion disease possible?

A

No

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9
Q

Repeats of which two amino acids at the N terminus of PrP are important for disease?

A

Glutamine and asparagine

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10
Q

What is the function of protein/RNA aggregation in a normal cell?

A

Overcoming stress. The aggregates are disassociated by heat shock proteins.

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11
Q

What neurons are most affected by protein aggregates in ALS?

A

Spinal motor proteins

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