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</p> Signup and view all the answers

    What is the role of Poh1 in aggresome formation?

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

    What mediates the clearance of aggresomes?

    <p>Lysosome</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</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</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</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</p> Signup and view all the answers

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

    <p>Acetylcholinesterase</p> Signup and view all the answers

    Which trinucleotide expansion is associated with Huntington's disease?

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

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

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

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

    <p>Macroautophagy</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</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</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</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</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</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</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</p> Signup and view all the answers

    What is an activated marker of inflammation in the brain?

    <p>Microglia</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</p> Signup and view all the answers

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

    <p>Neuroinflammation</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</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</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.</p> Signup and view all the answers

    What is the purpose of aggresome/inclusion bodies?

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

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

    <p>Alpha-synuclein</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.</p> Signup and view all the answers

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

    <p>Microscopy</p> Signup and view all the answers

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

    <p>SOD1</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</p> Signup and view all the answers

    Which trinucleotide expansion is associated with Huntington's disease?

    <p>CAG</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</p> Signup and view all the answers

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

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

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

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

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

    <p>Copper ion transport</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.</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.</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.</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.</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.</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.</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.</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</p> Signup and view all the answers

    Which genetic mutation can lead to synuclein release from exosomes?

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

    Which organelle is involved in the clearance of aggresomes?

    <p>Lysosome</p> Signup and view all the answers

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

    <p>P97</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</p> Signup and view all the answers

    What happens if there is trouble with aggresome clearance?

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

    What is the intermediate form between protein refolding and degradation?

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

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

    <p>CAG</p> Signup and view all the answers

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

    <p>Acetylcholinesterase</p> Signup and view all the answers

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

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

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

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

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

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

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