A gene that is often mutated in people with a neurodegenerative disease may help to keep immune responses in check.

Mutations in the C9ORF72 gene are commonly found in people with motor neuron disease, also known as amyotrophic lateral sclerosis (ALS), as well as in people with a form of dementia. To determine the function of the C9ORF72 protein, Kevin Eggan at Harvard University in Cambridge, Massachusetts, and his colleagues introduced mutations that diminished or eliminated the function of the equivalent protein in mice. The mutant mice had autoimmune disease, over-produced inflammatory molecules and died prematurely.

The authors conclude that C9ORF72 has an important role in controlling inflammation and autoimmunity.

Sci. Transl. Med. 8, 347ra93 (2016)