Protein fights traffic jams in ALS cells

February 17, 2016

Usually a cell works like a well-run city, but some diseases can gum up the works. One example is a form of the degenerative disease ALS caused by a mutation of the VAPB gene. This mutant gene produces abnormal proteins that stick together and cause traffic jams in the endoplasmic reticulum (ER), the place where certain proteins are made and shuttled to other parts of the cell. These traffic jams stress the ER, which triggers cell death in neurons that control muscle movement. Now, Dr. Johnny Ngseee and Angie Darbyson have discovered that a protein called ORP3 can act like a traffic cop in cell models of ALS, reducing the jams and restoring the flow of proteins. If it works the same way in animal studies, this discovery could possibly lead to drugs that could delay the onset of symptoms in this form of ALS. See Experimental Cell Research for details.

Funding: Canadian Institutes of Health Research (CIHR), Natural Sciences and Engineering Research Council of Canada (NSERC), The Ottawa Hospital Foundation

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