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Genetic studies indicate that amyotrophic lateral sclerosis is part of a spectrum of disorders, ranging from spinal muscular atrophy to frontotemporal dementia that share common pathological mechanisms. Amyotrophic lateral sclerosis Type 8 is a familial disease caused by mis-sense mutations in VAPB. VAPB is localized to the cytoplasmic surface of the endoplasmic reticulum, where it serves as a docking point for cytoplasmic proteins and mediates inter-organelle interactions with the endoplasmic reticulum membrane. A gene knock-in model of amyotrophic lateral sclerosis Type 8 based on the VapBP56S mutation and VapB gene deletion has been generated in rats. These animals display a range of age-dependent phenotypes distinct from those previously reported in mouse models of amyotrophic lateral sclerosis Type 8. A loss of motor neurones in VapBP56S\/+ and VapBP56S\/P56S animals is indicated by a reduction in the number of large choline acetyl transferase\u2013staining cells in the spinal cord. VapB\u2212\/\u2212 animals exhibit a relative increase in cytoplasmic TDP-43 levels compared with the nucleus, but no large protein aggregates. Concomitant with these spinal cord pathologies VapBP56S\/+, VapBP56S\/P56S and VapB\u2212\/\u2212 animals exhibit age-dependent changes in paw placement and exerted pressures when traversing a CatWalk apparatus, consistent with a somatosensory dysfunction. Extramotor dysfunction is reported in half the cases of motor neurone disease, and this is the first indication of an associated sensory dysfunction in a rodent model of amyotrophic lateral sclerosis. Different rodent models may offer complementary experimental platforms with which to understand the human disease.<\/jats:p>","DOI":"10.1093\/braincomms\/fcae184","type":"journal-article","created":{"date-parts":[[2024,5,24]],"date-time":"2024-05-24T23:26:50Z","timestamp":1716593210000},"source":"Crossref","is-referenced-by-count":4,"title":["Spinal cord neurone loss and foot placement changes in a rat knock-in model of amyotrophic lateral sclerosis Type 8"],"prefix":"10.1093","volume":"6","author":[{"given":"Brenda","family":"Murage","sequence":"first","affiliation":[{"name":"Centre for Discovery Brain Sciences, Edinburgh University , Edinburgh EH8 9XD , UK"},{"name":"Euan MacDonald Centre for MND Research, Edinburgh University , Edinburgh EH16 4SB , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han","family":"Tan","sequence":"additional","affiliation":[{"name":"Centre for Discovery Brain Sciences, Edinburgh University , Edinburgh EH8 9XD , 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