摘要
Amyotrophi c lateral s c lerosis(ALS)and frontotemporal dementia(FTD)are two closely related disorders with overlapping clinical,genetic,and neuropathological features,forming a continuous disease spectrum(Ling et al.,2013).The major pathological hallmark of ALS and FTD are the depletion from the nucleus of the RNA-binding proteins TAR DNA‐binding protein 43(TDP-43)and FUsed in Sarcoma(FUS)and their abnormal accumulation in ubiquitin-positive cytoplasmic inclusions(Ling et al.,2013).
基金
supported by AriSLA Foundation(MLOpathy and SUMOsolvable)
Banca d’Italia
German Research Foundation(DFG
WE 1406/16-1)
ALS Stichting grant“The Dutch ALS Tissue Bank”(to SC)。
