It is interesting to note that the electron-dense TDP-43 positive aggregates detected inside mitochondria in TDP-43 proteinopathy patient brain samples (Fig 1D) are reminiscent of the EM findings in lymphoblasts expressing LonP1 mutations of patients affected by cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome [74]. Here, LONP1 is linked to CODAS syndrome.