Recent studies have demonstrated that CHCHD10 is important for neuronal health.1 As such,CHCHD10 gene variants have been reported in patients with ALS, FTD, Parkinson’s disease, motor neuron disease, and mitochondrial myopathy, suggesting that they contribute to neurodegenerative disease progression.1,3,4 Additional work is needed to understand the underlying function and regulation of CHCHD10, in its native and mutant conformation, to advance the development of therapeutic strategies for targeting these deteriorating diseases. Here, CHCHD10 is linked to motor neuron disorder.