Prions Flashcards

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1
Q

Viroids

A

Very small, circular pieces of RNA that are infectious + pathogenic in plants; lack capsids

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2
Q

Prions

A

Proteinaceous Infective Particles

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3
Q

PrP

A

Normal mammalian cytoplasmic membrane protein

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4
Q

What is the structure of normal PrP?

A

Several alpha helices

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5
Q

Prion PrP

A

Folds into beta-pleated sheets despite having the same amino acid sequence

“like an accordion”

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6
Q

What does prion PrP do to normal PrP?

A

They turn them into beta-sheets (prion protein)

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7
Q

What are prions associated with?

A

Rare, progressive, neurodegenerative disorders

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8
Q

What are examples of disorders associated with prions?

A

Mad cow disease (bovine spongiform encephalitis)
Scrapie in sheep
Chronic wasting disease in deer + elk

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9
Q

Where does prion PrP aggregate?

A

The brain to form hard, insoluble clumps (amyloid plagues + fibrous structures)

  • Brain, spinal cord, eyes
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10
Q

How long does it take for symptoms of vCJD to show in humans?

A

10-20 years

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11
Q

What are examples of symptoms of vCJD?

A

Depression + anxiety, memory impairment, difficulty walking, confusion, fatigue, difficulty speaking

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12
Q

Sporadic transmission of vCJD

A

Random misfolding, occurs around age 60

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13
Q

Familial transmission of vCJD

A

Predisposition to misfolding, DNA-> AA’s

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14
Q

Foodborne transmission of vCJD

A

Eating infected beef

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15
Q

Medical pathways of transmitting vCJD

A

Surgery, transplants, transfusions

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16
Q

Variant Creutzfeldt-Jakob (vCJD)

A

Prion disease in humans

17
Q

What does prion protein effect?

A

Lymphoid tissue (spleen, tonsils, appendix, tonsils)

18
Q

How are prion proteins destroyed?

A

Incineration, autoclaving in concentrated sodium hydroxide soaking in 2 N NaOH or undiluted sodium hypochlorite for one hour, treating with keratinase + detergent at 100 degrees Celsius