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More than 88 genetic mutations have been associated with HSP, yet the mechanisms underlying these disorders are not well understood. We replicated the pathophysiology of one form of HSP known as spastic paraplegia 15 (SPG15) in zebrafish. This disorder is caused in humans by mutations in the\n                    <jats:italic>ZFYVE26<\/jats:italic>\n                    gene, which codes for a protein called SPASTIZIN. We show that, in zebrafish, the significant reduction of Spastizin caused degeneration of large motor neurons. Motor neuron degeneration is associated with axon demyelination in the spinal cord and impaired locomotion in the\n                    <jats:italic>spastizin<\/jats:italic>\n                    mutants. Our findings reveal that the reduction in Spastizin compromises axonal integrity and affects the myelin sheath, ultimately recapitulating the pathophysiology of HSPs.\n                  <\/jats:p>","DOI":"10.1098\/rsob.240100","type":"journal-article","created":{"date-parts":[[2024,11,6]],"date-time":"2024-11-06T06:12:07Z","timestamp":1730873527000},"update-policy":"https:\/\/doi.org\/10.1098\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Axon demyelination and degeneration in a zebrafish\n                    <i>spastizin<\/i>\n                    model of hereditary spastic paraplegia"],"prefix":"10.1098","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-4728-5400","authenticated-orcid":true,"given":"Vranda","family":"Garg","sequence":"first","affiliation":[{"name":"Department of Cellular Neurobiology, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Selina","family":"Andr\u00e9","sequence":"additional","affiliation":[{"name":"Department of Cellular Neurobiology, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luisa","family":"Heyer","sequence":"additional","affiliation":[{"name":"Department of Cellular Neurobiology, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gudrun","family":"Kracht","sequence":"additional","affiliation":[{"name":"Department of Developmental Biochemistry, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Torben","family":"Ruhwedel","sequence":"additional","affiliation":[{"name":"Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patricia","family":"Scholz","sequence":"additional","affiliation":[{"name":"Department of Plant Biochemistry, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Till","family":"Ischebeck","sequence":"additional","affiliation":[{"name":"Department of Plant Biochemistry, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"},{"name":"Institute of Plant Biology and Biotechnology, University of M\u00fcnster, M\u00fcnster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hauke B.","family":"Werner","sequence":"additional","affiliation":[{"name":"Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Dullin","sequence":"additional","affiliation":[{"name":"Institute for Diagnostic and Interventional Radiology, University Medical Center, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacob","family":"Engelmann","sequence":"additional","affiliation":[{"name":"Faculty of Biology, Bielefeld University, Bielefeld, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wiebke","family":"M\u00f6bius","sequence":"additional","affiliation":[{"name":"Department of Neurogenetics, Max Planck Institute for Multidisciplinary Sciences, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin C.","family":"G\u00f6pfert","sequence":"additional","affiliation":[{"name":"Department of Cellular Neurobiology, Georg-August-University G\u00f6ttingen, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roland","family":"Dosch","sequence":"additional","affiliation":[{"name":"Institute for Humangenetics, University Medical Center, G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1816-3241","authenticated-orcid":true,"given":"Bart R. 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