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Susan Lindquist interview: What happens when there is protein misfolding?

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Misfolding is happening all the time in cells. There is a very substantial fraction of proteins that misfold and never make it to the normal, folded state. The cell devotes a tremendous amount of energy trying to coax them back into their proper folds. If it cannot do that, if it cannot get the fold right, then it will degrade the protein, so most proteins that misfold wind up getting degraded. That’s kind of a waste of energy. It also means that if a particular protein has a mutation in it that won’t let it fold very well – – like the mutation that causes cystic fibrosis, that protein is hundreds of amino acids long, and it has just one mistake, just one mistake, one amino acid – and that protein misfolds, it winds up getting degraded. And that functionality that you need in the lung, it just disappears, and that’s what causes that terrible illness. The other side of protein misfolding that causes problems in biology... that sometimes if proteins misfold and they don’t get degraded, they hang around and they’re sticky and interact with other things... ...and they can make a mess, make a lot of problems. So the other kind of protein misfolding diseases that we get are not because of an absence of the protein because it gets degraded, but because the protein is misfolding and gumming up the works and sticking around, doing things that it shouldn’t be doing – interacting with membranes, for example, and causing membranes to be leaky. That’s another aspect of protein folding. Those are two kinds of ways in which protein misfolding can cause diseases. Protein folding problems are responsible for an enormous variety of human diseases. The fact that there are so many different diseases that are caused by problems with protein folding is our way of being able to see visually, and immediately in a way that matters to us, what all biological systems are facing all the time: every single protein in there can go off pathway – can misfold – at any one time, and environmental stresses cause lots of problems with protein folding. All biological systems are kind of poised at a position where they are so crowded... ...that they’re barely making the proteins that they should, and environmental stress can easily send that off pathway, or genetic mutations can send it off pathway, too.

Video Details

Duration: 2 minutes and 41 seconds
Country: Sweden
Language: English
Producer: MoleCluesTV
Director: Per Thorén
Views: 45
Posted by: locumele on May 12, 2014

Professor Susan Lindquist interviewed for MoleClues. Entire interview available on

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