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To investigate the impact of mislocalized TDP-43 on neuronal cell bodies, axons and axonal terminals, we utilized the mouse visual system to create a new model of TDP-43 proteinopathy. Mouse (C57BL\/6J) retinal ganglion cells (RGCs) were transduced with GFP-tagged human wild-type TDP-43 (hTDP-WT-GFP) and human TDP-43 with a mutation in the nuclear localization sequence (hTDP-\u0394NLS-GFP), to cause TDP-43 mislocalization, with \u223c60% transduction efficiency achieved. Expression of both hTDP-WT-GFP and hTDP-\u0394NLS-GFP resulted in changes to neurofilament expression, with cytoplasmic TDP-43 being associated with significantly (P&amp;lt;0.05) increased neurofilament heavy expression in the cell soma, and both forms of altered TDP-43 leading to significantly (P&amp;lt;0.05) decreased numbers of neurofilament-positive axons within the optic nerve. Alterations to neurofilament proteins were associated with significantly (P&amp;lt;0.05) increased microglial density in the optic nerve and retina. Furthermore, expression of hTDP-WT-GFP was associated with a significant (P&amp;lt;0.05) increase in pre-synaptic input into RGCs in the retina. The current study has developed a new model that allows detailed examination of alterations to TDP-43 and will contribute to the knowledge of TDP-43-mediated neuronal alterations and degeneration.<\/jats:p>","DOI":"10.1242\/dmm.047548","type":"journal-article","created":{"date-parts":[[2021,1,6]],"date-time":"2021-01-06T12:51:40Z","timestamp":1609937500000},"source":"Crossref","is-referenced-by-count":14,"title":["TDP-43 mislocalization drives neurofilament changes in a novel model of TDP-43 proteinopathy"],"prefix":"10.1242","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9846-7738","authenticated-orcid":false,"given":"Rachel","family":"Atkinson","sequence":"first","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Medical Science Precinct, 17 Liverpool Street, Hobart, Tasmania 7000, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacqueline","family":"Leung","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Medical Science Precinct, 17 Liverpool Street, Hobart, Tasmania 7000, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Bender","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Medical Science Precinct, 17 Liverpool Street, Hobart, Tasmania 7000, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew","family":"Kirkcaldie","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Medical Science Precinct, 17 Liverpool Street, Hobart, Tasmania 7000, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Vickers","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Medical Science Precinct, 17 Liverpool Street, Hobart, Tasmania 7000, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"King","sequence":"additional","affiliation":[{"name":"Wicking Dementia Research and Education Centre, University of Tasmania, Medical Science Precinct, 17 Liverpool Street, Hobart, Tasmania 7000, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2021,2,11]]},"reference":[{"key":"2021042623053885400_DMM047548C1","doi-asserted-by":"publisher","first-page":"1439","DOI":"10.1016\/j.visres.2004.12.015","article-title":"The optomotor response: a robust first-line visual screening method for mice","volume":"45","author":"Abdeljalil","year":"2005","journal-title":"Vision Res."},{"key":"2021042623053885400_DMM047548C2","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1016\/j.ophtha.2013.08.045","article-title":"Microcystic macular edema: retrograde maculopathy caused by optic neuropathy","volume":"121","author":"Abegg","year":"2014","journal-title":"Ophthalmology"},{"key":"2021042623053885400_DMM047548C3","doi-asserted-by":"publisher","first-page":"536","DOI":"10.1016\/j.neuron.2013.12.018","article-title":"Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations","volume":"81","author":"Alami","year":"2014","journal-title":"Neuron"},{"key":"2021042623053885400_DMM047548C4","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/0165-0270(95)00155-7","article-title":"Anterograde axonal tracing with the subunit B of cholera toxin: a highly sensitive immunohistochemical protocol for revealing fine axonal morphology in adult and neonatal brains","volume":"65","author":"Angelucci","year":"1996","journal-title":"J. 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