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Microbiology > Prion Diseases > Flashcards

Prion Diseases Flashcards

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Biology 101 flashcards
1
Q

Rare, fatal, rapidly progressive neurodegenerative diseases that occur in humans & other animal species

A

Prion diseases

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2
Q

In prion disease, a presence of _____ within the neuropil, produces a _____ appearance

A

Small vacuoles; spongiform

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3
Q

The complex net of axonal, dendritic & glial branching that forms the bulk of central nervous system gray matter of the brain & in which the nerve cell bodies are embedded:

A

Neuropil

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4
Q

Describe prion diseases:

A

-Rare
-Fatal
-Rapidly progressive
Neurodegenerative diseases that occur in humans & other animals

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5
Q

What animal prion diseases affects cattle?

A

Bovine, spongiform encephalopathy

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6
Q

What animal prion disease affects sheep & goats?

A

Scrapie

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7
Q

Human prion diseases include: (5)

A
  1. Kuru
  2. Creutzfeldt-Jakob disease (CJD)
  3. Variant Creutzfelft-Jakob disease (Variant CJD)
  4. Gerstmann-Straussler-Scheinker Syndrome
  5. Fatal familial insomnia (FFI)
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8
Q

Fatal neurodegenerative disease of sheep:

A

Scrapie

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9
Q

Scrapie is demonstrated to be a _____ disorder

A

Transmissible

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10
Q

Fatal neurodegenerative disease of humans recognized since the 1920, demonstrated to be transmissible in the 1960s (humans to chimpanzees):

A

Creutzfeldt-Jakob Disease (CJD)

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11
Q

What are the early symptoms of CJD?

A

-memory problems
-behavioral changes
-poor coordination
-visual disturbances

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12
Q

What are the layer symptoms of CJD?

A

-dementia
-involuntary movements
-blindness
-weakness
-coma

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13
Q

What is the outcome of CJD?

A

70% of people die within 1 year of diagnosis

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14
Q

It was discovered during the search for the infectious agents of Scrapie & CJD, that infection agents were _____ meaning not cellular, therefore, possibly a ____

A

Filterable; virus

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15
Q

When determining what the infectious material of Scrapie & CJD was, ionizing radiation did not affect transmission of Scrapie & CJD, what does this mean?

A

The infectious material did not contain DNA or RNA

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16
Q

Because ionizing radiation did not affect the transmission of Scrapie & CDJ, the hypothesis of the 1960s concluded that the scrapie & CJD are caused by:

A

Solely proteins

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17
Q

What term was created to describe Scrapie & CJD?

A

Prion

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18
Q

What term was created to describe Scrapie & CJD,

A
19
Q

The term prion means:

A

Proteinaceous infection

20
Q

The cellular protein involved in prions:

A

PrP (prion protein)

21
Q

Describe endogenous Prp:

A

Normal protein in the body that is NOT an disease agent

22
Q

Endogenous Prp’s can adopt a couple of different confirmations:

A
  1. Normal
  2. Disease forming
23
Q

The difference between a normal PrP & a disease-causing PrP occurs at the:

A

Confirmation level

24
Q

What is significant about an endogenous PrP converting to prion form?

A

It can continue to cause other endogenous PrPs to convert to the disease causing form

25
Q

What is responsible for encoding PrP protein?

A

PRNP gene on chromosome 20

26
Q

The PrP gene displays:

A

Polymorphism

27
Q

What do we mean when we say the PrP gene displays polymorphism?

A

Variance of gene sequence which results in variance of amino acid sequence

28
Q

Describe an area in the PrP sequence that we may see polymorphism:

A

Met or Val at codon 129 of PRNP gene

29
Q

What is the occurrence of seeing Met or Val at codon 129 of the PRNP gene:

A

Met= 60%
Val= 40%

30
Q

When an individual is homozygous for either Met or Val at codon 129 of the PRNP gene, what does this mean?

A

They are at increased risk of disease (CJD)

31
Q

The scrapie/prion form of Prp (PrPsc) is resistance to _____ & accumulates in ____

A

Degradation; Amyloid fibrils

32
Q

PrPc (normal form of PrP) is strongly expressed in both ____ & ____ of the CNS

A

Neurons & glial cells

33
Q

What is the function of PrPc in the CNS?

A

Appears to regulate ion channels & neurotransmitter receptors at the pre- & post-synaptic levels

34
Q

What is the median age of death for an individuals with CJD?

A

68 years

35
Q

What is the median age of death for an individual with a variant of CJD?

A

28

36
Q

What is the median duration of illness for an individual with CJD?

A

4-5 months

37
Q

What is the median duration of illness for an individual with a variant of CJD?

A

13-14 months

38
Q

What are the clinical signs & symptoms of CJD?

A

Dementia & early neurologic signs

39
Q

What are the clinical signs & symptoms of variant CJD?

A

Prominent psychiatric/behavioral symptoms; painful dysthesias; delayed neurologic signs

40
Q

Describe the accumulation of PrPSC in brain tissue in an individual with CJD compared to an individual with Variant CJD:

A

CJD: Variable accumulation

Variant CJD: Marked accumulation

41
Q

What is a clinical concern of prion diseases?

A

Prions may NOT be inactivated by means of routine surgical instrument sterilization procedures

42
Q

The WOH & US CDC and Prevention, recommend that instrumentation used during prion cases must be:

A

Immediately destroyed

43
Q

Secondary to destruction of instruments use in prion cases, it is recommended that ____ & ____ be used in combination to process instruments that come into contact with high-infectivity tissues:

A

Heat & chemical decontamination

44
Q

Since the adoption of current sterilization procedures regarding prion disease instrumentation use in 1976 what have we seen?

A

No cases of iatrogenic transmission of CJD that been reported

Microbiology (22 decks)

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