Neurons have a hidden self-defence against ALS — and a young researcher who lost family to the disease found it

Shohei Sakai came to Nagoya University in Japan specifically to hunt for an ALS cure, after the disease struck his own family. ALS is driven by a protein, TDP-43, that clumps inside the nerve cells controlling movement. Working with Koji Yamanaka's team, Sakai found that motor neurons carry a surprise weapon: a protein-shredding machine called the immunoproteasome, normally used by immune cells against viruses. When faulty TDP-43 appears, a sensor molecule sounds an alarm that switches the shredder on — in the affected cell and its neighbours — chopping the protein up before it can clump. The work, in EMBO Reports, points to a whole new route for treatments.

SDG 3 · Good health and well-being — Mechanism: tech innovation