CNS Protein Anomalies: Prions And Amyloids (Msq Drill 276)

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1. What is the leading cause of dementia?
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2. Bovine spongiform encephalopathy is commonly known as:
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3. Which of the prion proteins - Prpc or PrPsc forms multimeric aggregates and is protease resistant?
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4. When can the diagnosis of prion disease or alzheimer's disease be definitively made?
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5. New Guinea, Cannibalism of human brain, severe involvement of CEREBELLUM, extracellular plaques.
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6. Can cognitively intact elderly patients also exhibit senile plaques?
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7. PrPsc is more highly enriched in _______ . This  leads to its aggregations and resistance within the cell.
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8. Familial CJD is inherited in an __________ pattern.
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9. Prion diseases are also referred to as:
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10. Prion disease: mean age of onset was 26 y/o, longer duration of illness (12 months), sporadic cases. Exhibited more behavioral and sensory disturbances. No EEg findings.

What is the prion disease?
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11. Between alzheimers disease and CJD which one is accompanied by muscle twitching and loss of coordination?
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12. Do protein conformational diseases involve proteins that were initially normal or that were already malformed?
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13. What does amyloid "mistakenly" mean?
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14. Prion protein:
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15. Hyperphosphorylation of tau leads to its aggregation. these are known as ______________ and are found in Alzheimers disease.
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16. Can prion proteins become infectious?
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17. The process of prion protein propagation is exponential and _____ .
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18. The primary feature of protein conformational diseases is that of a normal cellular protein that becomes ________ and _______ .
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19. Beta-sheets typically have this quality:
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20. What cholesterol drug also reduces the levels of beta-amyloid in the brain?
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21. The animal prion disease found in sheep and goats is known as:
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22. What is the most common form of TSE in humans?
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23. The proteins that undergo conformational changes all involve what highly enriched protein?
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24. What is derived from protein/APP (amyloid precursor protein) and forms plaques in the brain?
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25. In which pathway  is the amyloid-B protein fragment derived from?
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26. Which of the following are the four main human forms of Prion diseses?
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27. The normal prion protein is constitutively expressed as a:
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28. Both _____ and _____ involve dementia.
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29. Is vacoulization seen intracellularly or extracellularly?

Explanation

Amyloid like proteins depositions are seen both intra and extra cellularly.

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30. When deposition of proteins occurs, a ________ protein arrangement is visible when stained with Congo Red dye.
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31. Most genetic diseases of amyloid aggregates are inherited in what pattern?
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32. Superoxide dismutase:
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33. In Alzheimers disease the tau protein is:
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34. Are infectious prions normally destroyed by sterilization?

Explanation

Prion proteins are extremely heat resistant, chemical and radiation resistant as well.

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35. Which of the following is a characteristic feature seen with CJD?

Explanation

Diagnosis is by ruling out other possiblities and EEG. Death is usually within 12 months of onset. Median illness duration is 4 months.

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36. Which of the prion diseases affects the cerebrum?
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37. B-Amyloid and tau are involved in the pathogenesis of Alzheimer's disease. Which of the two uses microtubules and is thus associated with intracellular accumulations?

Explanation

B-amyloid is primarily extracellular

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38. What histopathological finding is responsible for the spongiosis or spongiform degeneration of prion diseases?
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39. Gerstmann-Straussler-Scheinker syndrome is a familial prion disease with AD inheritance. Which of the following is characteristic of this disease?
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40. Smaller _________ attach to the oligomers and a seeding of the amyloids occurs.
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41. The early phase of amyloid seeding is called the:
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42. Presenilin is involved in:
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43. Which of the causes of CJD  shows age related somatic mutations of the PRNO or spontaneous conversion of PrPc into PrPsc?
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44. The amyoloid precursor protein is a _____________ highly enriched in neuronal cells.
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45. Amyloid like proteins are generally in their ________ form before they form aggregates.
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46. When the aggregates are forming they are known as:
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47. Tau is a microtubule assoicated protein (MAP) that has what function?
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48. CJD, Kuru and GSS all affect which part of the brain?
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49. Which of the following are neuropathological manifestions of Alzheimer's disease. Check all that apply.
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50. Which of the following is an immunotherapy that is used to stimulate a patient's immune system in order to target and degrade beta amyloid?
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51. Cell death in prion diseases is by apoptosis, therefore this is evidence that there is :
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52. Prions acquired though contaminated EEG electrodes or neurosurgical instruments, corneal transplants, dura mater grafts, human pituitary derived human growth hormone.This is classified as _______ .

Explanation

Most cases have been eliminated through proper decontamination or autoclave and screening guidelines.

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53. The amyloidogenic pathway requires what two enzymes? Check all that apply:
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54. Tarenflubril/R-flubiprofen are _________ used to reduce the formation of B-amyloid.
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55. The pathogenic form or prion protein is known as PrPsc -. Is the amino acid sequence of this pathogenic form different from the normal form?

Explanation

They both have the same amino acid sequence.

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56. The PrPsc monomers that are fomred are unstable, as a result they ________ in order to become stable.
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57. Which of the following are the three clinical halmarks of prion diseases? check all that apply.

Explanation

ataxia is loss of muscle coordination. myoclonus is involuntary muscle twitching.

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58. What is the predominant pathway that process APP? (amyloid precursor protein) 
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59. The change in conformations that proteins undergo in protein conformational diseases include which of the following: Check all that apply:
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60. Match the disease with the protein in the next 6 questions:

tau protein: Check all that apply
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61. The human prion gene is located on chromosome
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62. What is the average age of onset of CJD?

Explanation

several forms: heredity, sporadic, acquired

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63. The prion proteins primarily follow:
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64. **** What are the hallmark pathological lesions of Alzheimer's disease? Chek all that apply.
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65. Amyloidosis are deposited:

Explanation

Eg Alzheimers beta-amyloid deposition. Amyloid or amyloid-like proteins may be deposited systematically.

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66. What makes Fatal Familial Insomnia profoundly different from the other prion disease? Check all that apply.
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67. When do you typically start to see symptoms of amyloid deposition diseases?
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68. Which of the following are characteristics of prion diseases? Check all that apply.
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69. What are the three forms of prion diseases?
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70. Amyloid proteins refers to any number of proteins that are deposited in cells and share selected ___________ features.
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71. Do conformational diseases ALWAYS involve the deposition of altered protein?
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72. Which of the following are arguments that have been posed in favor of protein aggregation being the cause of effects of the diseases that are manifested. Check all that apply.
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73. Alpha-synuclein: Check all that apply.
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74. So if the pathogenic form of the prion protein has a similar amino acid sequence from the normal type, why is it disease causing? Check all that apply.

Explanation

this is what leads to its aggregation in the cell

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75. The PrPc or normal prion protein is primarily: Check all that apply.

Explanation

the pthogenic forms PrPsc self propagates in order to change the normal form into the abnormal form. The mechanism is poorly understood.

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76. In which of the following conditions is there a genetic link between APP (amyloid precursor protein) and alzheimers disease? Check all that apply.
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77. Which of the following are known routes of transmission of prion proteins?
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What is the leading cause of dementia?
Bovine spongiform encephalopathy is commonly known as:
Which of the prion proteins - Prpc or PrPsc forms multimeric...
When can the diagnosis of prion disease or alzheimer's...
New Guinea, Cannibalism of human brain, severe involvement of...
Can cognitively intact elderly patients also exhibit senile plaques?
PrPsc is more highly enriched in _______ . This  leads to its...
Familial CJD is inherited in an __________ pattern.
Prion diseases are also referred to as:
Prion disease: mean age of onset was 26 y/o, longer duration of...
Between alzheimers disease and CJD which one is accompanied by...
Do protein conformational diseases involve proteins that were...
What does amyloid "mistakenly" mean?
Prion protein:
Hyperphosphorylation of tau leads to its aggregation. these are known...
Can prion proteins become infectious?
The process of prion protein propagation is exponential and _____ .
The primary feature of protein conformational diseases is that of a...
Beta-sheets typically have this quality:
What cholesterol drug also reduces the levels of beta-amyloid in the...
The animal prion disease found in sheep and goats is known as:
What is the most common form of TSE in humans?
The proteins that undergo conformational changes all involve what...
What is derived from protein/APP (amyloid precursor protein) and...
In which pathway  is the amyloid-B protein fragment derived from?
Which of the following are the four main human forms of Prion diseses?
The normal prion protein is constitutively expressed as a:
Both _____ and _____ involve dementia.
Is vacoulization seen intracellularly or extracellularly?
When deposition of proteins occurs, a ________ protein arrangement is...
Most genetic diseases of amyloid aggregates are inherited in what...
Superoxide dismutase:
In Alzheimers disease the tau protein is:
Are infectious prions normally destroyed by sterilization?
Which of the following is a characteristic feature seen with CJD?
Which of the prion diseases affects the cerebrum?
B-Amyloid and tau are involved in the pathogenesis of Alzheimer's...
What histopathological finding is responsible for the spongiosis or...
Gerstmann-Straussler-Scheinker syndrome is a familial prion disease...
Smaller _________ attach to the oligomers and a seeding of the...
The early phase of amyloid seeding is called the:
Presenilin is involved in:
Which of the causes of CJD  shows age related somatic mutations...
The amyoloid precursor protein is a _____________ highly enriched in...
Amyloid like proteins are generally in their ________ form before they...
When the aggregates are forming they are known as:
Tau is a microtubule assoicated protein (MAP) that has what function?
CJD, Kuru and GSS all affect which part of the brain?
Which of the following are neuropathological manifestions of...
Which of the following is an immunotherapy that is used to stimulate a...
Cell death in prion diseases is by apoptosis, therefore this is...
Prions acquired though contaminated EEG electrodes or neurosurgical...
The amyloidogenic pathway requires what two enzymes? Check all that...
Tarenflubril/R-flubiprofen are _________ used to reduce the formation...
The pathogenic form or prion protein is known as PrPsc -. Is the...
The PrPsc monomers that are fomred are unstable, as a result they...
Which of the following are the three clinical halmarks of prion...
What is the predominant pathway that process APP? (amyloid precursor...
The change in conformations that proteins undergo in protein...
Match the disease with the protein in the next 6 questions:tau...
The human prion gene is located on chromosome
What is the average age of onset of CJD?
The prion proteins primarily follow:
**** What are the hallmark pathological lesions of Alzheimer's...
Amyloidosis are deposited:
What makes Fatal Familial Insomnia profoundly different from the other...
When do you typically start to see symptoms of amyloid deposition...
Which of the following are characteristics of prion diseases? Check...
What are the three forms of prion diseases?
Amyloid proteins refers to any number of proteins that are deposited...
Do conformational diseases ALWAYS involve the deposition of altered...
Which of the following are arguments that have been posed in favor of...
Alpha-synuclein: Check all that apply.
So if the pathogenic form of the prion protein has a similar amino...
The PrPc or normal prion protein is primarily: Check all that apply.
In which of the following conditions is there a genetic link...
Which of the following are known routes of transmission of prion...
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