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

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    What is the leading cause of dementia?

    • Parkinson's disease
    • Alzheimers disease
    • CJD
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  • 2. 

    Bovine spongiform encephalopathy is commonly known as:

    • Elephantisis

    • Mad cow disease

    • Scrapie

    • Transmissible mink encephalopathy

    Correct Answer
    A. Mad cow disease
  • 3. 

    Which of the prion proteins - Prpc or PrPsc forms multimeric aggregates and is protease resistant?

    • PrPsc

    • PrPc

    Correct Answer
    A. PrPsc
  • 4. 

    When can the diagnosis of prion disease or alzheimer's disease be definitively made?

    • By MRI

    • At autopsy

    • After a mental status exam

    • By CT scan

    Correct Answer
    A. At autopsy
  • 5. 

    New Guinea, Cannibalism of human brain, severe involvement of CEREBELLUM, extracellular plaques.

    • CJD

    • GSS

    • Kuru

    Correct Answer
    A. Kuru
  • 6. 

    Can cognitively intact elderly patients also exhibit senile plaques?

    • Yes

    • No

    Correct Answer
    A. Yes
  • 7. 

    PrPsc is more highly enriched in _______ . This  leads to its aggregations and resistance within the cell.

    • Beta pleated sheets

    • Alpha helice sheets

    Correct Answer
    A. Beta pleated sheets
  • 8. 

    Familial CJD is inherited in an __________ pattern.

    • Autosomal recessive

    • Autosomal dominant

    • X-linked recessive

    • X-linked dominant

    Correct Answer
    A. Autosomal dominant
  • 9. 

    Prion diseases are also referred to as:

    • Non-transmissible spongiform encephalopathies

    • Transmissible spongiform encephalopathies (TSE)

    • Ungulate spongiform encephalopathies

    • Chronic wasting diseases

    Correct Answer
    A. Transmissible spongiform encephalopathies (TSE)
  • 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?

    • CJD

    • New variant CJD AKA mad cow disease

    • Alzheimers disease

    • Huntington's disease

    Correct Answer
    A. New variant CJD AKA mad cow disease
  • 11. 

    Between alzheimers disease and CJD which one is accompanied by muscle twitching and loss of coordination?

    • Alzheimers

    • CJD

    Correct Answer
    A. CJD
  • 12. 

    Do protein conformational diseases involve proteins that were initially normal or that were already malformed?

    • Initially normal proteins

    • Initially abnormal proteins

    Correct Answer
    A. Initially normal proteins
  • 13. 

    Prion protein:

    • Prion disease

    • Alzheimers disease

    • Corticobasal degeneration

    • Frontotemporal dementia

    • ALS

    Correct Answer
    A. Prion disease
  • 14. 

    Hyperphosphorylation of tau leads to its aggregation. these are known as ______________ and are found in Alzheimers disease.

    • Cluster like lesions

    • Neurofibrillary tangles

    • Tau like entanglements

    • Tau inclusion bodies

    Correct Answer
    A. Neurofibrillary tangles
  • 15. 

    Can prion proteins become infectious?

    • No

    • Yes

    Correct Answer
    A. Yes
  • 16. 

    The process of prion protein propagation is exponential and _____ .

    • Autocrine

    • Autocatalytic

    • Exocrine

    • Exocatalytic

    Correct Answer
    A. Autocatalytic
  • 17. 

    What does amyloid "mistakenly" mean?

    • Starch like

    • Protein like

    • Zinc like

    • Amino acid like

    Correct Answer
    A. Starch like
  • 18. 

    The primary feature of protein conformational diseases is that of a normal cellular protein that becomes ________ and _______ .

    • Misfolded

    • Aggregated

    • Denatured

    • Compartmentalized

    Correct Answer(s)
    A. Misfolded
    A. Aggregated
  • 19. 

    Beta-sheets typically have this quality:

    • They are soluble

    • They are insoluble

    • They are flexible

    • They are easily denatured

    Correct Answer
    A. They are insoluble
  • 20. 

    What cholesterol drug also reduces the levels of beta-amyloid in the brain?

    • Simvastatin

    • Lipitor

    • Rovastatin

    • Nystantin

    Correct Answer
    A. Simvastatin
  • 21. 

    The animal prion disease found in sheep and goats is known as:

    • Mad cow disease

    • Scrapie

    • Chronic wasting disease

    • Feline spongiform encephalopathy

    Correct Answer
    A. Scrapie
  • 22. 

    What is the most common form of TSE in humans?

    • GSS

    • Alzheimers disease

    • Creutzfeldt-Jacob Disease

    • Fatal familial insomnia

    • Kuru

    Correct Answer
    A. Creutzfeldt-Jacob Disease
  • 23. 

    The proteins that undergo conformational changes all involve what highly enriched protein?

    • Alpha helix

    • B-pleated sheets

    • Beta-2-microglobulin

    • Beta-trace proteins

    Correct Answer
    A. B-pleated sheets
  • 24. 

    What is derived from protein/APP (amyloid precursor protein) and forms plaques in the brain?

    • Beta- amyloid

    • Alpha helix

    • Beta-2-microgloblulin

    Correct Answer
    A. Beta- amyloid
  • 25. 

    In which pathway  is the amyloid-B protein fragment derived from?

    • Amyloidogenic pathway

    • Non-amyloidogenic pathway

    Correct Answer
    A. Amyloidogenic pathway
  • 26. 

    Which of the following are the four main human forms of Prion diseses?

    • Creutzfeldt-jakob disease (CJD)

    • Mad cow disease

    • Gerstmann-Straussler-Scheinker disease (GSS)

    • Fatal familial insomnia (FFI)

    • Kuru

    Correct Answer(s)
    A. Creutzfeldt-jakob disease (CJD)
    A. Gerstmann-Straussler-Scheinker disease (GSS)
    A. Fatal familial insomnia (FFI)
    A. Kuru
  • 27. 

    The normal prion protein is constitutively expressed as a:

    • Cytoplasmic body

    • Membrane glycoprotein

    • Vacoule

    • Endosome

    • Plasmid

    Correct Answer
    A. Membrane glycoprotein
  • 28. 

    Both _____ and _____ involve dementia.

    • Alzheimers disease

    • GSS

    • CJD

    Correct Answer(s)
    A. Alzheimers disease
    A. CJD
  • 29. 

    Is vacoulization seen intracellularly or extracellularly?

    • Intracellularly

    • Extracellulary

    Correct Answer
    A. Intracellularly
    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.

    • Microtubular

    • Circular

    • Fibrillar

    • Whirlpool like

    Correct Answer
    A. Fibrillar
  • 31. 

    Most genetic diseases of amyloid aggregates are inherited in what pattern?

    • Autosomal recessive

    • X-linked recessive

    • X-linked dominant

    • Autosomal dominant

    Correct Answer
    A. Autosomal dominant
  • 32. 

    Superoxide dismutase:

    • Amyotropic lateral sclerosis

    • Alzheimers disease

    • Lewy body dementia

    • Prion disease

    Correct Answer
    A. Amyotropic lateral sclerosis
  • 33. 

    In Alzheimers disease the tau protein is:

    • Dephosphorylated

    • Hyperphosphorylated

    • Denatured

    • Hydrolyzed

    Correct Answer
    A. Hyperphosphorylated
  • 34. 

    Are infectious prions normally destroyed by sterilization?

    • Yes

    • no

    Correct Answer
    A. no
    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?

    • Slow progressing dementia

    • Rapidly progressive dementia

    • No dementia

    Correct Answer
    A. Rapidly progressive dementia
    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?

    • CJD

    • GSS

    • Parkinson's disease

    Correct Answer
    A. CJD
  • 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?

    • B-amyloid

    • Tau

    Correct Answer
    A. Tau
    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?

    • Membrane glycoproteins

    • Vacoulized cells

    • Neurofibrillary tangles

    • Whirlpool like cell structure

    Correct Answer
    A. Vacoulized cells
  • 39. 

    Gerstmann-Straussler-Scheinker syndrome is a familial prion disease with AD inheritance. Which of the following is characteristic of this disease?

    • Dementia with no cerebellar involvement

    • Spinocerebellar ataxia with dementia

    Correct Answer
    A. Spinocerebellar ataxia with dementia
  • 40. 

    Smaller _________ attach to the oligomers and a seeding of the amyloids occurs.

    • Dimers

    • Monomers

    • Trimeric aggregates

    • Amyloid aggregates

    Correct Answer
    A. Monomers
  • 41. 

    The early phase of amyloid seeding is called the:

    • Log phase

    • Lag phase

    • Growth phase

    • Termination phaseh

    Correct Answer
    A. Lag phase
  • 42. 

    Presenilin is involved in:

    • APP processing

    • Amyloid deposition

    • Neurofibril formation

    Correct Answer
    A. APP processing
  • 43. 

    Which of the causes of CJD  shows age related somatic mutations of the PRNO or spontaneous conversion of PrPc into PrPsc?

    • Iatrogenic CJD

    • Sporadic CJD

    • Familial CJD

    Correct Answer
    A. Sporadic CJD
  • 44. 

    The amyoloid precursor protein is a _____________ highly enriched in neuronal cells.

    • Cytoplasmic protein

    • Membrane glycoprotein

    • Extracellular protein

    • Nuclear protein

    Correct Answer
    A. Membrane glycoprotein
  • 45. 

    Amyloid like proteins are generally in their ________ form before they form aggregates.

    • Dimeric

    • Trimeric

    • Aggregrate

    • Monomeric

    Correct Answer
    A. Monomeric
  • 46. 

    When the aggregates are forming they are known as:

    • Dimers

    • Clusters

    • Oligomers

    Correct Answer
    A. Oligomers
  • 47. 

    Tau is a microtubule assoicated protein (MAP) that has what function?

    • To stabilize synaptic terminals

    • To stabilize neuronal microtubules

    • To facilitate neuronal transport

    • To facilitate endocytosis

    Correct Answer
    A. To stabilize neuronal microtubules
  • 48. 

    CJD, Kuru and GSS all affect which part of the brain?

    • Cerebrum

    • Cerebellum

    • Cortex

    • Brainstem

    • Limbic system

    Correct Answer
    A. Cerebellum
  • 49. 

    Which of the following are neuropathological manifestions of Alzheimer's disease. Check all that apply.

    • Extracellular amyloid plaques

    • Extracellular neurofibrillary tangles from tau accumulation

    • Intracellular neurofibrillary tangles from tau accumulation

    • Intracellular amylod plaques

    Correct Answer(s)
    A. Extracellular amyloid plaques
    A. Intracellular neurofibrillary tangles from tau accumulation

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  • Jul 06, 2022
    Quiz Edited by
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  • Mar 03, 2010
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