ALS and Dementia


Anchor lead: What do ALS and a common form of dementia have in common? Elizabeth Tracey reports

ALS and a common form of dementia known as ‘frontotemporal’ share a defect, research by Jeffrey Rothstein, an ALS expert at Johns Hopkins, and colleagues has shown. It’s a place inside cells where materials are transported into the cell’s center, or nucleus. They do so via an opening called a pore, Rothstein explains.

Rothstein: What we learned is that of these 30 different proteins that make up the pore we literally analyzed almost every single one of them and we said well which one is being screwed up? We learned that eight of them seemed to drop out of the pore, the pore is still there but they’re missing elements so think of a Lego complex with a hole here, a hole there. And that affects transport through it. So why are they missing? Turns out what we learn is that one of them is like the domino, you take that one away and the pore disassembles. And conversely if you put that one back in, it reassembles, which no one had done before.  :31

Rothstein says the proteins that make up the pore usually remain in the cell for years, so they are also of interest in aging. At Johns Hopkins, I’m Elizabeth Tracey.