Researchers discover how cells clear misfolded proteins from tissues
Could this apply to Kennedy's?
Location: Chicago, IL
I doubt it. It is about the process of clearing misfolded proteins that are outside of cells by bringing them into cells where organelles can disassemble them. In our case the misfolded proteins are mostly in the nucleus of the cell and rely on different mechanisms to exit into the cytoplasm for recycling.
Cam, that paper is interesting and thanks for sharing it. But as ToddAllen pointed out, it's about proteins outside the cell, and how they are brought in to be destroyed.
In KD the mutant proteins are inside the cell, so the process described in the paper won't work for them. Instead, they should be destroyed through one of two processes.
The first one is called the ubiquitin proteasome system or UPS for short. There is a sort of "tag" called ubiquitin that is normally attached to the mutant protein by certain enzymes, and the ubiquitin "tag" marks the protein for destruction by the proteasome. In KD the UPS doesn't work properly, for reasons that are not fully understood, so the unwanted protein is not destroyed.
The other process is called autophagy, and it doesn't work KD either, also for reasons that are not fully understood.
This paper contains a discussion of the UPS and autophagy in KD.
Scroll down to the sections "Ubiquitin Proteasome System Impairment" and "Autophagy" to read more about them.
For the basics, Wikipedia has articles on "Ubiquitin" and "Autophagy" that are helpful.This message has been edited. Last edited by: Dan B,
Kennedy's Disease Association
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