Proper disposal of mis-functioning proteins and aging cellular components is ver

Question

Proper disposal of mis-functioning proteins and aging cellular components is very important, when these processes do not work properly, cellular functions can be severely impaired. Choose a protein involved in managing misfolded proteins in the ER and a protein involved in proper disposal of components in the lysosome. For each compare how it is specifically designed and regulated for its particular function. For one of these, describe what happens in the cell if this regulation does not function properly.

Explanation / Answer

The protein quality system (PQS) in the ER monitors protein folding and eliminates misfolded proteins through ER associated protein degradation (ERAD).

Accumulation of different forms of misfolded proteins can lead to alteration of protein homeostasis and cause extensive cell and tissue damage.

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