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Evidence suggests that ALS is a \u2018dying-back\u2019 disease, with peripheral denervation and axonal degeneration occurring before loss of motor neuron cell bodies. Distal to a nerve injury, a similar pattern of axonal degeneration can be seen, which is mediated by an active axon destruction mechanism called Wallerian degeneration. Sterile alpha and TIR motif-containing 1 (\n                    <jats:italic>Sarm1<\/jats:italic>\n                    ) is a key gene in the Wallerian pathway and its deletion provides long-term protection against both Wallerian degeneration and Wallerian-like, non-injury induced axonopathy, a retrograde degenerative process that occurs in many neurodegenerative diseases where axonal transport is impaired. Here, we explored whether Sarm1 signalling could be a therapeutic target for ALS by deleting\n                    <jats:italic>Sarm1<\/jats:italic>\n                    from a mouse model of ALS-FTD, a TDP-43\n                    <jats:sup>Q331K<\/jats:sup>\n                    , YFP-H double transgenic mouse.\n                    <jats:italic>Sarm1<\/jats:italic>\n                    deletion attenuated motor axon degeneration and neuromuscular junction denervation. Motor neuron cell bodies were also significantly protected. Deletion of\n                    <jats:italic>Sarm1<\/jats:italic>\n                    also attenuated loss of layer V pyramidal neuronal dendritic spines in the primary motor cortex. Structural MRI identified the entorhinal cortex as the most significantly atrophic region, and histological studies confirmed a greater loss of neurons in the entorhinal cortex than in the motor cortex, suggesting a prominent FTD-like pattern of neurodegeneration in this transgenic mouse model. Despite the reduction in neuronal degeneration,\n                    <jats:italic>Sarm1<\/jats:italic>\n                    deletion did not attenuate age-related behavioural deficits caused by TDP-43\n                    <jats:sup>Q331K<\/jats:sup>\n                    . However,\n                    <jats:italic>Sarm1<\/jats:italic>\n                    deletion was associated with a significant increase in the viability of male TDP-43\n                    <jats:sup>Q331K<\/jats:sup>\n                    mice, suggesting a detrimental role of Wallerian-like pathways in the earliest stages of TDP-43\n                    <jats:sup>Q331K<\/jats:sup>\n                    -mediated neurodegeneration. Collectively, these results indicate that anti-SARM1 strategies have therapeutic potential in ALS-FTD.\n                  <\/jats:p>","DOI":"10.1186\/s40478-019-0800-9","type":"journal-article","created":{"date-parts":[[2019,10,28]],"date-time":"2019-10-28T13:05:08Z","timestamp":1572267908000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":89,"title":["Sarm1 deletion suppresses TDP-43-linked motor neuron degeneration and cortical spine loss"],"prefix":"10.1186","volume":"7","author":[{"given":"Matthew A.","family":"White","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ziqiang","family":"Lin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugene","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher M.","family":"Henstridge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emiliano","family":"Pena Altamira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camille K.","family":"Hunt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ella","family":"Burchill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isobel","family":"Callaghan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Loreto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heledd","family":"Brown-Wright","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Mead","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camilla","family":"Simmons","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Cash","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P.","family":"Coleman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8682-6844","authenticated-orcid":false,"given":"Jemeen","family":"Sreedharan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,10,28]]},"reference":[{"key":"800_CR1","doi-asserted-by":"publisher","first-page":"402","DOI":"10.1093\/brain\/awn312","volume":"132","author":"R Adalbert","year":"2009","unstructured":"Adalbert R, Nogradi A, Babetto E, Janeckova L, Walker SA, Kerschensteiner M, Misgeld T, Coleman MP (2009) Severely dystrophic axons at amyloid plaques remain continuous and connected to viable cell bodies. 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