Protein Folding Disorders Flashcards

1
Q

What are the components of the cellular quality control system?

A

Proteasomes

Autophagy

ERAD (ER-associated degradation).

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2
Q

What are some abnormalities in protein folding?

A

Improper degradation

Improper localization

Dominant negative mutations

Gain-of-toxic function

Amyloid accumulation

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3
Q

You are studying protein folding and examine a group of cells that have overactive cellular degradation systems. What is the result of this?

A

mutant, misfolded, incomplete degraded proteins.

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4
Q

Incorrectly folded proteins lead to improper subcellular localization

A

loss-of-function

gain of function toxicity

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5
Q

What is a key modulator in protein homeostasis?

A

molecular chaperons

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6
Q

How does a mutant protein affect the function of wild-type protein?

A

It causes a loss of protein activity and interferes with the function of the protein at cellular and structural levels.

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7
Q

Protein conformational changes cause dominant phenotypes. What is the result of this?

A

Toxic proteins are made.

APOE4 disrupts mitochondrial function.

Superoxide dismutase is present

SRC kinases in cancer.

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8
Q

What are amyloid fibers?

A

Insoluble protein aggregates.

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9
Q

What is the sequence of amyloidogenic proteins?

A

VQIVY

It causes amyloid-related diseases

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10
Q

What do lower order oligomers cause?

A

Toxic effects.

Amyloid deposits could be a protective mechanism

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11
Q

What kind of structure do several amyloidogenic proteins form?

A

A pore like structure.

This disrupts the cell membrane integrity.

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12
Q

In what individuals are misfolded forms of the protein frequently observed in?

A

The elderly

Those with mutations in the protein early in life.

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13
Q

How do amyloids progess to amyloid plaques?

A

Seeding

Fibril formation

Deposit

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14
Q

What is a potential remediation process of amyloid buildup?

A

Blocking aggregate formation.

THis can be done with antibodies that recognize conformational changes and specific sequences.

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15
Q

How does hormetic stress affect an individual?

A

Applying moderate levels of stress could trigger beneficial and adaptive stress defense pathways, allowing longer life.

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16
Q

What is proteostasis?

A

Maintenance of protein homoeostasis.

17
Q

What does proteostasis require?

A

Protein production

Folding

Degradation

18
Q

What are unfolded protein responses?

A

Complex pathways to ensure proteostasis in different compartments.

They are found in the cytosol, ER and mitochondria

19
Q

When is the apoptotic pathway used?

A

It is used last; it is the last line of defense.

20
Q

What is heat shock repsonse?

A

A response that manages denatured proteins in the cytosol.

21
Q

What are the two major mitochondrial chaperone systems?

A

mtHSP70

Multimeric protein HSP60 - HSP10

22
Q

How many proteins of the ETC are encoded by mtDNA?

A

13

23
Q

What are protein quality control proteases?

A

They recognize and degrade the proteins that do not fold.

They are also properly assembled.

24
Q

What is the functio of UPRmt?

A

It senses the overload of the QC system capacity.

It activates the transcription of nuclear encoded protective genes and re-establishes the mitochondrial homeostasis.

25
Q

What is a limitation of the UPRmt signaling?

A

The signal transmission from the unfolded/misfolded mitochondrial proteins to the nucleus cannot be accomplished using HSR and UPR system.

26
Q

What system assists in signal transmission from the unfolded/misfolded mitochondrial proteins to the nucleus?

A

retrograde response