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TDP-43 pathology has also been found in brain tissues under non-amyotrophic lateral sclerosis conditions, suggesting mechanistic links between TDP-43-related amyotrophic lateral sclerosis and various neurological disorders. This study aimed to assess TDP-43 pathology in the spinal cord motor neurons of tauopathies. We examined 106 spinal cords from consecutively autopsied cases with progressive supranuclear palsy (n\u2009=\u200926), corticobasal degeneration (n\u2009=\u200912), globular glial tauopathy (n\u2009=\u20095), Alzheimer\u2019s disease (n\u2009=\u200921) or Pick's disease (n\u2009=\u20096) and neurologically healthy controls (n\u2009=\u200936). Ten of the progressive supranuclear palsy cases (38%) and seven of the corticobasal degeneration cases (58%) showed mislocalization and cytoplasmic aggregation of TDP-43 in spinal cord motor neurons, which was prominent in the cervical cord. TDP-43 aggregates were found to be skein-like, round-shaped, granular or dot-like and contained insoluble C-terminal fragments showing blotting pattern of amyotrophic lateral sclerosis or frontotemporal lobar degeneration. The lower motor neurons also showed cystatin-C aggregates, although Bunina bodies were absent in haematoxylin-eosin staining. The spinal cord TDP-43 pathology was often associated with TDP-43 pathology of the primary motor cortex. Positive correlations were shown between the severities of TDP-43 and four-repeat (4R)-tau aggregates in the cervical cord. TDP-43 and 4R-tau aggregates burdens positively correlated with microglial burden in anterior horn. TDP-43 pathology of spinal cord motor neuron did not develop in an age-dependent manner and was not found in the Alzheimer\u2019s disease, Pick's disease, globular glial tauopathy and control groups. Next, we assessed SFPQ expression in spinal cord motor neurons; SFPQ is a recently identified regulator of amyotrophic lateral sclerosis\/frontotemporal lobar degeneration pathogenesis, and it is also reported that interaction between SFPQ and FUS regulates splicing of MAPT exon 10. Immunofluorescent and proximity-ligation assays revealed altered SFPQ\/FUS-interactions in the neuronal nuclei of progressive supranuclear palsy, corticobasal degeneration and amyotrophic lateral sclerosis-TDP cases but not in Alzheimer\u2019s disease, Pick's disease and globular glial tauopathy cases. Moreover, SFPQ expression was depleted in neurons containing TDP-43 or 4R-tau aggregates of progressive supranuclear palsy and corticobasal degeneration cases. Our results indicate that progressive supranuclear palsy and corticobasal degeneration may have properties of systematic motor neuron TDP-43 proteinopathy, suggesting mechanistic links with amyotrophic lateral sclerosis-TDP. SFPQ dysfunction, arising from altered interaction with FUS, may be a candidate of the common pathway.<\/jats:p>","DOI":"10.1093\/brain\/awac091","type":"journal-article","created":{"date-parts":[[2022,3,9]],"date-time":"2022-03-09T12:21:02Z","timestamp":1646828462000},"page":"2769-2784","source":"Crossref","is-referenced-by-count":37,"title":["Motor neuron TDP-43 proteinopathy in progressive supranuclear palsy and corticobasal degeneration"],"prefix":"10.1093","volume":"145","author":[{"given":"Yuichi","family":"Riku","sequence":"first","affiliation":[{"name":"Institute for Medical Science of Aging, Aichi Medical University , Aichi , Japan"},{"name":"Department of Neurology, Graduate School of Nagoya University , Aichi , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Iwasaki","sequence":"additional","affiliation":[{"name":"Institute for Medical Science of Aging, Aichi Medical University , Aichi , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9555-0659","authenticated-orcid":false,"given":"Shinsuke","family":"Ishigaki","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Nagoya University , Aichi , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akio","family":"Akagi","sequence":"additional","affiliation":[{"name":"Institute for Medical Science of Aging, Aichi Medical University , Aichi , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7415-8159","authenticated-orcid":false,"given":"Masato","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Tokyo Metropolitan Institute of Medical Science , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenya","family":"Nishioka","sequence":"additional","affiliation":[{"name":"Department of Neurology, Juntendo University School of Medicine , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuanzhe","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Neurology, Juntendo University School of Medicine , Tokyo , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miho","family":"Riku","sequence":"additional","affiliation":[{"name":"Department of Pathology, Aichi Medical University , Aichi , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8828-8085","authenticated-orcid":false,"given":"Takeshi","family":"Ikeuchi","sequence":"additional","affiliation":[{"name":"Department of Molecular Genetics, Brain Research Institute, Niigata University , Niigata , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Fujioka","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Nagoya University , Aichi , 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