Protein Folding

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Western University
Anatomy and Cell Biology
Anatomy and Cell Biology 4429A
Paul Walton

Chaperones allow proteins to fold 13 of the proteins that are folded do not fold properly and have to be disposed of Hsp70 delivers proteins to the lysosomes or proteosomes if you are a misfolded proteins A small fraction of proteins can fold on their own and some fold with the help of a molecular chaperone Some proteins dont do any of one of these three things pictured in the diagram and find themselves aggregated together in insoluble clumps Cells are not very good at not very good atunfolding proteins It is so dangerous to unfold proteinsWhat happens if I make a protein that does not fold properly Its function has been lost The pathway this protein is involved with will be rerouted to a different pathway or will stop The protein could also do something new which might be toxic to the cell or undergo a gain of function Accumulationif there is a mutation and this protein isnt targeted to be destroyed these proteins might accumulate They could also expose domains to interact with other proteins and begin to aggregate The cell will try and recover from aggregation by sending it to the proteosome or the lysosome however these dont usually work Aggregation is almost always an irreversible process As far as diseases go they can be organized into these main categories Cystic fibrosisprotein that is unable to fold Proteins might also not be able to hold their form in the case of an unstable protein In the case of Alzheimers disease proteins are toxic because of a gain of function This list is now much longer Lisa Hannan wrote a paper about all the diseases we
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