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The toxic substrate that accumulates in this disease, psychosine, induces alterations in membrane lipid rafts with downstream consequences to cellular signaling pathways that include impaired protein kinase C, ERK, and AKT\u2010glycogen synthase kinase\u20103\u03b2 (GSK3\u03b2) activation. In addition to impaired recruitment of signaling proteins to lipid rafts, endocytosis and axonal transport are affected in KD. Defects in AKT\u2010GSK3\u03b2 activation, a central pathway regulating microtubule stability, together with alterations in neurofilaments and microtubules and severely defective axonal transport, highlight the importance of the neuronal cytoskeleton in KD. This Review critically discusses these primary neuronal defects as well as new windows for action opened by their identification that may contribute to effectively correct the neuropathology that underlies this disorder. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.23771","type":"journal-article","created":{"date-parts":[[2016,9,17]],"date-time":"2016-09-17T11:22:31Z","timestamp":1474111351000},"page":"1037-1041","source":"Crossref","is-referenced-by-count":10,"title":["Axonal pathology in <scp>K<\/scp>rabbe's disease: The cytoskeleton as an emerging therapeutic target"],"prefix":"10.1002","volume":"94","author":[{"given":"Joana","family":"Nogueira\u2010Rodrigues","sequence":"first","affiliation":[{"name":"Nerve Regeneration Group, Instituto de Biologia Molecular e Celular (IBMC) and Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade Universidade do Porto Porto 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