ARCA due to mutations in coenzyme Q8A (COQ8A; formerly: ADCK3; now also referred to as ATX‐COQ8A2) might serve as a particularly promising candidate disease, as its underlying molecular pathogenesis includes aberrant coenzyme Q10 (CoQ10) synthesis, which might be susceptible to drug treatments, eg, CoQ10 supplementation.3 Here, COQ8A is linked to autosomal recessive cerebellar ataxia.