Interestingly, thirteen genes contain prioritised variants in both the WES and WGS datasets (ATP5F1A, CLPX, DNM1L, LARS2, NIT1, OGG1, OPA1, OXPHOS, PC, PCCB, PDHX, PDK2, and PREPL, Figure 5a,b) encoded proteins directly interact with the three mitochondrial sirtuins (SIRT3, SIRT4, and SIRT5), highlighting gene variants that were enriched in specific genes in ALS patients’ tissues. Here, PDHX is linked to amyotrophic lateral sclerosis.