Protein Aggregation & Proteasomal Function Quiz
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Questions and Answers

Which of the following is true about Q-bodies?

  • Q-bodies are a protective response to misfolded proteins (correct)
  • Q-bodies are the final form of protein degradation
  • Q-bodies are formed during protein refolding
  • Q-bodies are primarily found in neuronal orglial cell types

What is the purpose of aggresome/inclusion bodies?

  • To promote protein refolding
  • To transport misfolded proteins to the nucleus
  • To protect misfolded proteins (correct)
  • To degrade misfolded proteins

Which protein unloads polyubiquitination from aggregated proteins?

  • TRIM5O
  • P97 (correct)
  • LC3
  • Poh1

What happens to aggregated proteins during retrograde transport?

<p>They join onto growing aggresomes (C)</p> Signup and view all the answers

What is the role of Poh1 in aggresome formation?

<p>It removes K-63 polyUb (D)</p> Signup and view all the answers

What mediates the clearance of aggresomes?

<p>Lysosome (A)</p> Signup and view all the answers

What happens if there is trouble with aggresome clearance?

<p>The aggregate remains with LC3-P62-Ub interaction (C)</p> Signup and view all the answers

Which protein is associated with the formation of cytoplasmic inclusions called Lewy bodies in Parkinson's disease?

<p>Alpha-synuclein (D)</p> Signup and view all the answers

Which genetic mutation is responsible for causing 5-10% of Parkinson's disease cases?

<p>Alpha-synuclein mutation (A)</p> Signup and view all the answers

Which protein is associated with the formation of intraneuronal neurofibrillary tangles and extracellular amyloid plaques in Alzheimer's disease?

<p>Tau protein (A)</p> Signup and view all the answers

Which enzyme is targeted by cholinesterase inhibitors in the treatment of Alzheimer's disease?

<p>Acetylcholinesterase (B)</p> Signup and view all the answers

Which trinucleotide expansion is associated with Huntington's disease?

<p>CAG (B)</p> Signup and view all the answers

Which protein is associated with about 20% of familial cases of amyotrophic lateral sclerosis (ALS)?

<p>Superoxide dismutase (SOD1) (A)</p> Signup and view all the answers

Which intracellular protein degradation pathway involves the use of the p62 adapter protein and the lysosome?

<p>Macroautophagy (D)</p> Signup and view all the answers

Which protein is the main aggregating protein in inclusion bodies in many neurodegenerative diseases?

<p>a-synuclein (A)</p> Signup and view all the answers

What is the proposed link between a-synuclein oligomers, pore formation, UPS inhibition, and PD pathogenesis?

<p>a-synuclein oligomers cause pore formation which leads to UPS inhibition and PD pathogenesis (B)</p> Signup and view all the answers

What is the technique used to measure very small structures such as protein aggregates?

<p>Atomic force microscopy (C)</p> Signup and view all the answers

Which type of autophagy involves membrane formation around organelles and their engulfment by lysosomes?

<p>Macroautophagy (D)</p> Signup and view all the answers

What is the role of SUMO-1 modification of proteins in neurodegeneration?

<p>SUMO-1 modification promotes aggregate clearance (B)</p> Signup and view all the answers

How do metal ions, such as calcium, copper, and zinc, contribute to neurodegeneration?

<p>Metal ions cause calcium overload (A)</p> Signup and view all the answers

What is the role of ATP7a/b pump in neurodegenerative diseases?

<p>ATP7a/b pump regulates copper levels in cells (D)</p> Signup and view all the answers

Which of the following is a key feature of the cell-to-cell spread of alpha-synuclein in PD and MSA?

<p>Extracellular alpha-synuclein can be taken up by cells (B)</p> Signup and view all the answers

What is the potential role of exosomes in the spread of aggregate protein?

<p>Exosomes can carry alpha-synuclein and contribute to the spread of aggregate protein (A)</p> Signup and view all the answers

How can PARK9 mutations contribute to synuclein release from exosomes?

<p>PARK9 mutations enhance the release of synuclein from exosomes (C)</p> Signup and view all the answers

What is one of the links between neuroinflammation and neurodegeneration in AD and MSA?

<p>Neuroinflammation leads to an increase in neurodegeneration (A)</p> Signup and view all the answers

What is an activated marker of inflammation in the brain?

<p>Microglia (A)</p> Signup and view all the answers

How can the use of NSAIDs potentially lessen the risk of AD?

<p>NSAIDs can reduce neuroinflammation and decrease the risk of AD (A)</p> Signup and view all the answers

What can cause an increased expression of the cytoskeletal protein GFAP in the CNS?

<p>Neuroinflammation (B)</p> Signup and view all the answers

Which of the following is true about the cell-to-cell spread of alpha-synuclein?

<p>It can occur through extracellular uptake by cells (B)</p> Signup and view all the answers

What is the potential role of exosomes in the spread of alpha-synuclein in PD and MSA?

<p>Exosomes can carry alpha-synuclein and contribute to its spread (B)</p> Signup and view all the answers

Which of the following is true about Q-bodies?

<p>Q-bodies are the intermediate form between protein refolding and degradation. (B)</p> Signup and view all the answers

What is the purpose of aggresome/inclusion bodies?

<p>To protect misfolded proteins from degradation. (D)</p> Signup and view all the answers

What is the role of P62 adapter protein in aggresome formation?

<p>P62 adapter protein interacts with LC3 which has a membrane anchor. (A)</p> Signup and view all the answers

What is the main aggregating protein in inclusion bodies in many neurodegenerative diseases?

<p>Alpha-synuclein (D)</p> Signup and view all the answers

What is the proposed link between a-synuclein oligomers, pore formation, UPS inhibition, and PD pathogenesis?

<p>a-synuclein oligomers cause UPS inhibition which leads to pore formation and PD pathogenesis. (A)</p> Signup and view all the answers

What is the technique used to measure very small structures such as protein aggregates?

<p>Microscopy (A)</p> Signup and view all the answers

Which protein is associated with about 20% of familial cases of amyotrophic lateral sclerosis (ALS)?

<p>SOD1 (C)</p> Signup and view all the answers

Which protein is associated with the formation of cytoplasmic inclusions called Lewy bodies in Parkinson's disease?

<p>Alpha-synuclein protein (B)</p> Signup and view all the answers

Which trinucleotide expansion is associated with Huntington's disease?

<p>CAG (C)</p> Signup and view all the answers

Which protein is associated with the formation of intraneuronal neurofibrillary tangles and extracellular amyloid plaques in Alzheimer's disease?

<p>Tau protein (B)</p> Signup and view all the answers

Which enzyme is targeted by cholinesterase inhibitors in the treatment of Alzheimer's disease?

<p>Acetylcholinesterase (D)</p> Signup and view all the answers

Which protein is associated with about 20% of familial cases of amyotrophic lateral sclerosis (ALS)?

<p>Superoxide dismutase (SOD1) (D)</p> Signup and view all the answers

What is the technique used to measure very small structures such as protein aggregates?

<p>Electron microscopy (D)</p> Signup and view all the answers

What is the role of ATP7a/b pump in neurodegenerative diseases?

<p>Copper ion transport (D)</p> Signup and view all the answers

Which of the following is a proposed link between alpha-synuclein oligomers, pore formation, UPS inhibition, and PD pathogenesis?

<p>Alpha-synuclein oligomers induce UPS inhibition by blocking proteasomal function. (D)</p> Signup and view all the answers

What is the proposed link between metal ions, such as calcium, copper, zinc, and neurodegeneration?

<p>Copper misregulation causes raised copper levels in CSF, resulting in neurodegeneration. (D)</p> Signup and view all the answers

What is the role of SUMO-1 modification of proteins in neurodegeneration?

<p>Inhibiting SUMOylation can enhance aggregate clearance. (B)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein mutants in PD and CMA?

<p>Alpha-synuclein mutants inhibit CMA by interacting with Tsg101 and Vps4. (C)</p> Signup and view all the answers

What is the role of autophagy in clearing polyQ aggregates?

<p>Enhanced autophagy upregulates proteasome function, promoting clearance of polyQ aggregates. (D)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein and neurotransmitter vesicles?

<p>Alpha-synuclein interacts with SNARE complex in vesicle fusion. (B)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein and the 19s cap of the proteasome?

<p>Alpha-synuclein aggregates bind to the 19s cap, inhibiting proteasomal function. (C)</p> Signup and view all the answers

Which of the following is true about the cell-to-cell spread of alpha-synuclein in Parkinson's disease (PD) and Multiple System Atrophy (MSA)?

<p>Extracellular alpha-synuclein can be taken up by a cell (D)</p> Signup and view all the answers

Which genetic mutation can lead to synuclein release from exosomes?

<p>PARK9 mutations (C)</p> Signup and view all the answers

What is the role of neuroinflammation in neurodegenerative diseases, particularly Alzheimer's disease (AD) and Multiple System Atrophy (MSA)?

<p>Neuroinflammation can cause an increased expression of cytoskeletal protein GFAP (D)</p> Signup and view all the answers

Which of the following can lessen the risk of Alzheimer's disease (AD)?

<p>Use of nonsteroidal anti-inflammatory drugs (NSAIDs) (C)</p> Signup and view all the answers

What is the potential role of exosomes in the spread of alpha-synuclein in Parkinson's disease (PD) and Multiple System Atrophy (MSA)?

<p>Exosomes can carry synuclein and contribute to the spread of aggregate protein (C)</p> Signup and view all the answers

What is the key feature of the cell-to-cell spread of alpha-synuclein in Parkinson's disease (PD) and Multiple System Atrophy (MSA)?

<p>Extracellular alpha-synuclein can be taken up by a cell (D)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein oligomers, pore formation, UPS inhibition, and Parkinson's disease (PD) pathogenesis?

<p>Alpha-synuclein oligomers cause pore formation, leading to UPS inhibition and PD pathogenesis (C)</p> Signup and view all the answers

How do metal ions, such as calcium, copper, and zinc, contribute to neurodegeneration?

<p>Metal ions can induce protein aggregation and oxidative stress, contributing to neurodegeneration (B)</p> Signup and view all the answers

What can cause an increased expression of the cytoskeletal protein GFAP in the central nervous system (CNS)?

<p>Neuroinflammation (D)</p> Signup and view all the answers

Which of the following is the correct sequence of events in aggresome formation and degradation?

<p>Active transport to MTOC -&gt; Protein refolding -&gt; Protein degradation (B)</p> Signup and view all the answers

Which organelle is involved in the clearance of aggresomes?

<p>Lysosome (D)</p> Signup and view all the answers

Which protein is involved in the unloading of polyubiquitinated proteins from aggresomes?

<p>P97 (A)</p> Signup and view all the answers

What is the role of the P62 adapter protein in aggresome formation?

<p>Mediates the interaction between LC3 and the aggregate (B)</p> Signup and view all the answers

What happens if there is trouble with aggresome clearance?

<p>The aggregate remains in the cytoplasm (D)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein mutants and aggresome formation?

<p>Alpha-synuclein mutants impair active transport to MTOC (A)</p> Signup and view all the answers

What is the intermediate form between protein refolding and degradation?

<p>Q-bodies (C)</p> Signup and view all the answers

Which protein is associated with cytoplasmic inclusions called Lewy bodies in Parkinson's disease?

<p>Alpha-synuclein protein (D)</p> Signup and view all the answers

What is the main aggregating protein in intraneuronal neurofibrillary tangles and extracellular amyloid plaques in Alzheimer's disease?

<p>Tau protein (B)</p> Signup and view all the answers

What is the trinucleotide expansion associated with Huntington's disease?

<p>CAG (A)</p> Signup and view all the answers

Which enzyme is targeted by cholinesterase inhibitors in the treatment of Alzheimer's disease?

<p>Acetylcholinesterase (B)</p> Signup and view all the answers

What is the role of PINK1 mutation in Parkinson's disease?

<p>Binds to depolarized mitochondria (C)</p> Signup and view all the answers

Which protein is associated with about 20% of familial cases of amyotrophic lateral sclerosis (ALS)?

<p>Superoxide dismutase (SOD1) (A)</p> Signup and view all the answers

What is the main mechanism of intracellular protein degradation via the ubiquitin-proteasome system (UPS)?

<p>Proteasomal degradation (A)</p> Signup and view all the answers

Which of the following is a key feature of macroautophagy?

<p>Membrane forms around organelle and then engulfed by lysosome (C)</p> Signup and view all the answers

What is the proposed link between SUMO-1 modification of proteins and neurodegeneration?

<p>SUMOylation may promote aggregate clearance (A)</p> Signup and view all the answers

What is the proposed link between metal ions and neurodegeneration?

<p>AB peptide can directly activate AMPA receptors (A)</p> Signup and view all the answers

What is the role of ATP7a/b pump in neurodegenerative diseases?

<p>Regulates copper levels in cells (C)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein mutants in PD and CMA?

<p>Inhibit CMA by disrupting protein unfolding (B)</p> Signup and view all the answers

What is the proposed link between a-synuclein oligomers and UPS inhibition?

<p>Bind to the 19s cap of the proteasome and inhibit its function (A)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein and neurotransmitter vesicles?

<p>Interferes with binding to lipid vesicles in aggregated form (B)</p> Signup and view all the answers

Which of the following is a key feature of the cell-to-cell spread of alpha-synuclein in Parkinson's disease (PD) and Multiple System Atrophy (MSA)?

<p>Alpha-synuclein can move from cell to cell (B)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein mutants in PD and CMA?

<p>Alpha-synuclein mutants impair the function of chaperone-mediated autophagy (CMA) (A)</p> Signup and view all the answers

What is the role of Poh1 in aggresome formation?

<p>Poh1 is involved in the degradation of misfolded proteins in aggresomes (B)</p> Signup and view all the answers

What is the main aggregating protein in inclusion bodies in many neurodegenerative diseases?

<p>Tau protein (D)</p> Signup and view all the answers

What is the proposed link between alpha-synuclein oligomers, pore formation, UPS inhibition, and PD pathogenesis?

<p>Alpha-synuclein oligomers cause pore formation, leading to UPS inhibition and PD pathogenesis (D)</p> Signup and view all the answers

How can the use of NSAIDs potentially lessen the risk of AD?

<p>NSAIDs reduce neuroinflammation, which is linked to AD (A)</p> Signup and view all the answers

What is the potential role of exosomes in the spread of alpha-synuclein in PD and MSA?

<p>Exosomes can carry alpha-synuclein and contribute to its spread between cells (C)</p> Signup and view all the answers

Which protein is associated with the formation of cytoplasmic inclusions called Lewy bodies in Parkinson's disease?

<p>Alpha-synuclein (C)</p> Signup and view all the answers

What is the role of SUMO-1 modification of proteins in neurodegeneration?

<p>SUMO-1 modification of proteins can protect against neurodegeneration (B)</p> Signup and view all the answers

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