CNS Protein Anomalies: Prions And Amyloids (Msq Drill 276)
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- 1.
The primary feature of protein conformational diseases is that of a normal cellular protein that becomes ________ and _______ .
- A.
Misfolded
- B.
Aggregated
- C.
Denatured
- D.
Compartmentalized
Correct Answer(s)
A. Misfolded
B. Aggregated -
- 2.
Do protein conformational diseases involve proteins that were initially normal or that were already malformed?
- A.
Initially normal proteins
- B.
Initially abnormal proteins
Correct Answer
A. Initially normal proteins -
- 3.
The change in conformations that proteins undergo in protein conformational diseases include which of the following: Check all that apply:
- A.
Protein aggregations
- B.
Resistance to degradation
- C.
Abnormal accumulations
- D.
Protein denaturation
- E.
Cell toxicity
Correct Answer(s)
A. Protein aggregations
B. Resistance to degradation
C. Abnormal accumulations
E. Cell toxicity -
- 4.
Do conformational diseases ALWAYS involve the deposition of altered protein?
- A.
Yes
- B.
No
Correct Answer
A. Yes -
- 5.
The proteins that undergo conformational changes all involve what highly enriched protein?
- A.
Alpha helix
- B.
B-pleated sheets
- C.
Beta-2-microglobulin
- D.
Beta-trace proteins
Correct Answer
B. B-pleated sheets -
- 6.
Beta-sheets typically have this quality:
- A.
They are soluble
- B.
They are insoluble
- C.
They are flexible
- D.
They are easily denatured
Correct Answer
B. They are insoluble -
- 7.
When deposition of proteins occurs, a ________ protein arrangement is visible when stained with Congo Red dye.
- A.
Microtubular
- B.
Circular
- C.
Fibrillar
- D.
Whirlpool like
Correct Answer
C. Fibrillar -
- 8.
Amyloid proteins refers to any number of proteins that are deposited in cells and share selected ___________ features.
- A.
Conformational
- B.
Histologic
- C.
Symptomatologic
- D.
Laboratory
Correct Answer
D. Laboratory -
- 9.
What does amyloid "mistakenly" mean?
- A.
Starch like
- B.
Protein like
- C.
Zinc like
- D.
Amino acid like
Correct Answer
A. Starch like -
- 10.
Amyloidosis are deposited:
- A.
In the blood stream
- B.
In the PNS
- C.
In an organ
- D.
In the CNS
Correct Answer
C. In an organExplanation
Eg Alzheimers beta-amyloid deposition. Amyloid or amyloid-like proteins may be deposited systematically.Rate this question:
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- 11.
Amyloid like proteins are generally in their ________ form before they form aggregates.
- A.
Dimeric
- B.
Trimeric
- C.
Aggregrate
- D.
Monomeric
Correct Answer
D. Monomeric -
- 12.
When the aggregates are forming they are known as:
- A.
Dimers
- B.
Clusters
- C.
Oligomers
Correct Answer
C. Oligomers -
- 13.
Smaller _________ attach to the oligomers and a seeding of the amyloids occurs.
- A.
Dimers
- B.
Monomers
- C.
Trimeric aggregates
- D.
Amyloid aggregates
Correct Answer
B. Monomers -
- 14.
The early phase of amyloid seeding is called the:
- A.
Log phase
- B.
Lag phase
- C.
Growth phase
- D.
Termination phaseh
Correct Answer
B. Lag pHase -
- 15.
When do you typically start to see symptoms of amyloid deposition diseases?
- A.
As soon as conformational changes occur
- B.
When the fibrillar deposits are visible
- C.
As soon as aggregation commenses
- D.
When 70% deposition has taken place
Correct Answer
B. When the fibrillar deposits are visible -
- 16.
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.
- A.
Overexpression or mutated forms of the protein causing disease
- B.
New and emerging illnessess
- C.
Genetic associations
- D.
Co-localization of protein with cell loss
Correct Answer(s)
A. Overexpression or mutated forms of the protein causing disease
C. Genetic associations
D. Co-localization of protein with cell loss -
- 17.
Most genetic diseases of amyloid aggregates are inherited in what pattern?
- A.
Autosomal recessive
- B.
X-linked recessive
- C.
X-linked dominant
- D.
Autosomal dominant
Correct Answer
D. Autosomal dominant -
- 18.
Match the disease with the protein in the next 6 questions:tau protein: Check all that apply
- A.
Alzheimers
- B.
Parkinson's disease
- C.
Amyotropic lateral sclerosis
- D.
Pick's disease, progressive supranuclear palsy, corticobasal degeneration, frontotemporal dementia
Correct Answer(s)
A. Alzheimers
D. Pick's disease, progressive supranuclear palsy, corticobasal degeneration, frontotemporal dementia -
- 19.
Superoxide dismutase:
- A.
Amyotropic lateral sclerosis
- B.
Alzheimers disease
- C.
Lewy body dementia
- D.
Prion disease
Correct Answer
A. Amyotropic lateral sclerosis -
- 20.
Alpha-synuclein: Check all that apply.
- A.
Prion disease
- B.
Parkinson's disease
- C.
Huntington's disease
- D.
Lewy body dementia
Correct Answer(s)
B. Parkinson's disease
D. Lewy body dementia -
- 21.
Prion protein:
- A.
Prion disease
- B.
Alzheimers disease
- C.
Corticobasal degeneration
- D.
Frontotemporal dementia
- E.
ALS
Correct Answer
A. Prion disease -
- 22.
What is the leading cause of dementia?
- A.
Parkinson's disease
- B.
Alzheimers disease
- C.
CJD
Correct Answer
B. Alzheimers disease -
- 23.
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?
- A.
B-amyloid
- B.
Tau
Correct Answer
B. TauExplanation
B-amyloid is primarily extracellularRate this question:
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- 24.
What is derived from protein/APP (amyloid precursor protein) and forms plaques in the brain?
- A.
Beta- amyloid
- B.
Alpha helix
- C.
Beta-2-microgloblulin
Correct Answer
A. Beta- amyloid -
- 25.
Can cognitively intact elderly patients also exhibit senile plaques?
- A.
Yes
- B.
No
Correct Answer
A. Yes -
- 26.
Tau is a microtubule assoicated protein (MAP) that has what function?
- A.
To stabilize synaptic terminals
- B.
To stabilize neuronal microtubules
- C.
To facilitate neuronal transport
- D.
To facilitate endocytosis
Correct Answer
B. To stabilize neuronal microtubules -
- 27.
In Alzheimers disease the tau protein is:
- A.
Dephosphorylated
- B.
Hyperphosphorylated
- C.
Denatured
- D.
Hydrolyzed
Correct Answer
B. HyperpHospHorylated -
- 28.
Hyperphosphorylation of tau leads to its aggregation. these are known as ______________ and are found in Alzheimers disease.
- A.
Cluster like lesions
- B.
Neurofibrillary tangles
- C.
Tau like entanglements
- D.
Tau inclusion bodies
Correct Answer
B. Neurofibrillary tangles -
- 29.
Which of the following are neuropathological manifestions of Alzheimer's disease. Check all that apply.
- A.
Extracellular amyloid plaques
- B.
Extracellular neurofibrillary tangles from tau accumulation
- C.
Intracellular neurofibrillary tangles from tau accumulation
- D.
Intracellular amylod plaques
Correct Answer(s)
A. Extracellular amyloid plaques
C. Intracellular neurofibrillary tangles from tau accumulation -
- 30.
The amyoloid precursor protein is a _____________ highly enriched in neuronal cells.
- A.
Cytoplasmic protein
- B.
Membrane glycoprotein
- C.
Extracellular protein
- D.
Nuclear protein
Correct Answer
B. Membrane glycoprotein -
- 31.
What is the predominant pathway that process APP? (amyloid precursor protein)
- A.
Amyloidogenic pathway
- B.
non-amyloid pathway
- C.
Precursory pathway
- D.
Glycosylation
Correct Answer
B. non-amyloid pathway -
- 32.
The amyloidogenic pathway requires what two enzymes? Check all that apply:
- A.
Alpha-secretase
- B.
B-secretase
- C.
Amyloidase
- D.
Gamma-secretase
Correct Answer(s)
B. B-secretase
D. Gamma-secretase -
- 33.
In which pathway is the amyloid-B protein fragment derived from?
- A.
Amyloidogenic pathway
- B.
Non-amyloidogenic pathway
Correct Answer
A. Amyloidogenic pathway -
- 34.
In which of the following conditions is there a genetic link between APP (amyloid precursor protein) and alzheimers disease? Check all that apply.
- A.
Trisomy 21
- B.
Presenilin gene mutations
- C.
Early onset Alzheirmers disease
- D.
Late onset Alzheimers disease
- E.
Familial Alzheimers disease
Correct Answer(s)
A. Trisomy 21
B. Presenilin gene mutations
C. Early onset Alzheirmers disease -
- 35.
Presenilin is involved in:
- A.
APP processing
- B.
Amyloid deposition
- C.
Neurofibril formation
Correct Answer
A. APP processing -
- 36.
Tarenflubril/R-flubiprofen are _________ used to reduce the formation of B-amyloid.
- A.
Alpha secretase inhibitors
- B.
Amyloidase inhibitors
- C.
Gamma-secretase inhibitors
- D.
Beta-secretase inhibitors
Correct Answer
C. Gamma-secretase inhibitors -
- 37.
What cholesterol drug also reduces the levels of beta-amyloid in the brain?
- A.
Simvastatin
- B.
Lipitor
- C.
Rovastatin
- D.
Nystantin
Correct Answer
A. Simvastatin -
- 38.
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?
- A.
Pro-B amyloid vaccination
- B.
Anti-B amyloid vaccines
- C.
Pro-APP vaccination
- D.
Anti-APP vaccination
Correct Answer
B. Anti-B amyloid vaccines -
- 39.
Prion diseases are also referred to as:
- A.
Non-transmissible spongiform encephalopathies
- B.
Transmissible spongiform encephalopathies (TSE)
- C.
Ungulate spongiform encephalopathies
- D.
Chronic wasting diseases
Correct Answer
B. Transmissible spongiform encepHalopathies (TSE) -
- 40.
Can prion proteins become infectious?
- A.
No
- B.
Yes
Correct Answer
B. Yes -
- 41.
Which of the following are characteristics of prion diseases? Check all that apply.
- A.
Always inherited
- B.
Rapidly progressive
- C.
Fatal neurodegenerative disorders
- D.
Never inherited
- E.
Long incubation periods
Correct Answer(s)
B. Rapidly progressive
C. Fatal neurodegenerative disorders
E. Long incubation periods -
- 42.
What are the three forms of prion diseases?
- A.
Acute
- B.
Infectious
- C.
Inherited
- D.
Subacute
- E.
Spontaneous
Correct Answer(s)
B. Infectious
C. Inherited
E. Spontaneous -
- 43.
Which of the following are the four main human forms of Prion diseses?
- A.
Creutzfeldt-jakob disease (CJD)
- B.
Mad cow disease
- C.
Gerstmann-Straussler-Scheinker disease (GSS)
- D.
Fatal familial insomnia (FFI)
- E.
Kuru
Correct Answer(s)
A. Creutzfeldt-jakob disease (CJD)
C. Gerstmann-Straussler-Scheinker disease (GSS)
D. Fatal familial insomnia (FFI)
E. Kuru -
- 44.
The animal prion disease found in sheep and goats is known as:
- A.
Mad cow disease
- B.
Scrapie
- C.
Chronic wasting disease
- D.
Feline spongiform encephalopathy
Correct Answer
B. Scrapie -
- 45.
Bovine spongiform encephalopathy is commonly known as:
- A.
Elephantisis
- B.
Mad cow disease
- C.
Scrapie
- D.
Transmissible mink encephalopathy
Correct Answer
B. Mad cow disease -
- 46.
The human prion gene is located on chromosome
- A.
18
- B.
20
- C.
17
- D.
11
- E.
5
Correct Answer
B. 20 -
- 47.
The normal prion protein is constitutively expressed as a:
- A.
Cytoplasmic body
- B.
Membrane glycoprotein
- C.
Vacoule
- D.
Endosome
- E.
Plasmid
Correct Answer
B. Membrane glycoprotein -
- 48.
The pathogenic form or prion protein is known as PrPsc -. Is the amino acid sequence of this pathogenic form different from the normal form?
- A.
Yes
- B.
No
Correct Answer
B. NoExplanation
They both have the same amino acid sequence.Rate this question:
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- 49.
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.
- A.
It becomes mutated in situ
- B.
It has a different conformation
- C.
It takes over the machinery of the nucleus
- D.
It has different patterns of glycosylation
Correct Answer(s)
B. It has a different conformation
D. It has different patterns of glycosylationExplanation
this is what leads to its aggregation in the cellRate this question:
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- 50.
PrPsc is more highly enriched in _______ . This leads to its aggregations and resistance within the cell.
- A.
Beta pleated sheets
- B.
Alpha helice sheets
Correct Answer
A. Beta pleated sheets -
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