Astrocytes regulate axonal mitochondrial transport deficits in C9ORF72 amyotrophic lateral sclerosis motor neurons.

Astrocytes with C9ORF72 mutation impair axonal mitochondrial transport in ALS motor neurons, but boosting astrocytic bioenergetics restores function.

• Why it matters: Understanding how astrocyte dysfunction contributes to ALS progression is crucial, as disrupted mitochondrial transport affects neuron survival and disease severity.
• What they did: Researchers used human-induced pluripotent stem-cell-derived astrocytes with the C9ORF72 mutation in coculture with motor neurons and applied gene correction and bioenergetic enhancement techniques.
• The result: Restoring astrocytic mitochondrial function rescued axonal transport deficits, highlighting astrocytes' non-cell-autonomous role in maintaining neuronal health in ALS.