CHCHD10 and motor neuron disorder: Heterozygous mutations in CHCHD10 were first linked to motor neuron disease and frontotemporal dementia, myopathy and hyperkalemia, impaired respiratory chain function and multiple mtDNA deletions in the skeletal muscle in a large family of French origin, and in a Spanish family (none of the family members underwent muscle or skin biopsy) in 2014 [251].