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The precise extent and mechanistic basis of non\u2010motor neuron damage in human ALS and ALS animal models remain however unclear. To address this, we here studied progressive motor neuronopathy (<jats:italic>pmn<\/jats:italic>) mice carrying a missense loss\u2010of\u2010function mutation in tubulin binding cofactor E (TBCE). These mice manifest a particularly aggressive form of motor axon dying back and display a microtubule loss, similar to that induced by human ALS\u2010linked TUBA4A mutations. Using whole nerve confocal imaging of <jats:italic>pmn<\/jats:italic> \u00d7 thy1.2\u2010YFP16 fluorescent reporter mice and electron microscopy, we demonstrate axonal discontinuities, bead\u2010like spheroids and ovoids in <jats:italic>pmn<\/jats:italic> suralis nerves indicating prominent sensory neuropathy. The axonal alterations qualitatively resemble those in phrenic motor nerves but do not culminate in the loss of myelinated fibers. We further show that the <jats:italic>pmn<\/jats:italic> mutation decreases the level of TBCE, impedes microtubule polymerization in dorsal root ganglion (DRG) neurons and causes progressive loss of microtubules in large and small caliber suralis axons. Live imaging of axonal transport using GFP\u2010tagged tetanus toxin C\u2010fragment (GFP\u2010TTC) demonstrates defects in microtubule\u2010based transport in <jats:italic>pmn<\/jats:italic> DRG neurons, providing a potential explanation for the axonal alterations in sensory nerves. This study unravels sensory neuropathy as a pathological feature of mouse <jats:italic>pmn<\/jats:italic>, and discusses the potential contribution of cytoskeletal defects to sensory neuropathy in human motor neuron disease.<\/jats:p>","DOI":"10.1111\/bpa.12422","type":"journal-article","created":{"date-parts":[[2016,8,4]],"date-time":"2016-08-04T07:17:07Z","timestamp":1470295027000},"page":"459-471","source":"Crossref","is-referenced-by-count":17,"title":["Sensory neuropathy in progressive motor neuronopathy <i>(pmn)<\/i> mice is associated with defects in microtubule polymerization and axonal transport"],"prefix":"10.1111","volume":"27","author":[{"given":"Michael K.","family":"Sch\u00e4fer","sequence":"first","affiliation":[{"name":"Department of Anesthesiology and Research Center Translational Neurosciences University Medical Center of the Johannes Gutenberg\u2010University Mainz  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