Isolated peripheral lymphocytes also present aberrant mitochondria which can be correlated with clinical hallmarks of FXTAS [140,141] and postmortem brains of FXTAS patients mirrored the decrease in the levels of mature proteins such as Sodium/potassium-transporting ATPase subunit β (ATPB), Manganese superoxide dismutase (MnSOD) and frataxin found in skin fibroblasts [135,137]. This evidence concerns the gene SOD2 and fragile X-associated tremor/ataxia syndrome.