… development of TDP-43 proteinopathy from cytoplasmic … (4) FUS mutations are thought
to cause ALS, independent of … FTD cases are of unknown etiology but are linked by TDP-43 …

… in most cases of FTLD-TDP, but not FTLD-FUS or SOD1 … of TDP-43 may be involved in
FTLD-TDP and sporadic ALS and non-SOD1 FALS, but that this may not be the case in FTLD-FUS…

… In comparison, FTLD-TDP is less heterogeneous but still has … , not all TDP-43 proteinopathies
are FTLD-TDP, and TDP-43 … accepted that FTLD cases feature either TDP-43 or FUS or tau …

… in TDP-43/FUS proteinopathy in order to better understand the pathogenesis of ALS/FTLD-U. …
of SGs, as well as our hypothetical model of TDP-43 and FUS proteinopathy. Full-length …

… are implicated in ALS proteinopathy. Disease-causing mutations in FUS and TDP-43 cause
… affect FUS and TPD-43 localization can generate a ‘stressed out’ neuronal pathology and …

… Although mutations in TDP-43 and FUS/TLS have been reported only in ALS and FTLD
patients, TDP-43 and FUS/TLS immunoreactive inclusions have been observed in the nucleus …

… In conclusion, although mutations in TDP-43 and FUS are quite rare in ALS/FTLD patients,
they certainly seem to play a role in the pathology and could theoretically be used as a target …

… role in the pathogenesis of FTLD/ALS-related disorders. TDP-43 and FUS/TLS are typically
… been observed in ALS and FTLD patients with TDP-43 proteinopathy, demonstrating that …

… The clinical phenotypes of ALS and FTLD-TDP (FTLD with … and animal models of TDP-43
proteinopathy, and their relationship … into the emerging picture of TDP-43 proteinopathy, and to …

… /FUS interaction has a disease specificity for ALS/FTLD-TDP and 4R-tauopathies. SFPQ,
FUS, and TDP-43 … SFPQ and FUS are components of the core structure in para-speckle body, …