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Mutations in <jats:italic>copper\u2010zinc superoxide dismutase 1<\/jats:italic> (<jats:italic>SOD1<\/jats:italic>) are a known genetic cause of ALS, and the <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> mouse has been used extensively to investigate molecular mechanisms in ALS. In recent years, evidence suggests that ALS and frontotemporal dementia form a spectrum disorder ranging from motor to cognitive dysfunctions. Thus, we tested male and female <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> mice for the first time before the onset of debilitating motor impairments in behavioural domains relevant to both ALS and frontotemporal dementia. <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> males displayed reduced locomotion, exploration and increased anxiety\u2010like behaviours compared with control males. Intermediate\u2010term spatial memory was impaired in <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> females, whereas long\u2010term spatial memory deficits as well as lower acoustic startle response, and prepulse inhibition were identified in <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> mice of both sexes compared with respective controls. Interestingly, <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> males exhibited an increased conditioned cue <jats:italic>freezing<\/jats:italic> response. <jats:italic>Nosing<\/jats:italic> behaviours were also elevated in both male and female <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> when assessed in social paradigms. In conclusion, <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> mice exhibit a variety of sex\u2010specific behavioural deficits beyond motor impairments supporting the notion of an ALS\u2010frontotemporal spectrum disorder. Thus, <jats:italic>SOD1<\/jats:italic><jats:sup>\n<jats:italic>G93A<\/jats:italic><\/jats:sup> mice may represent a useful model to test the efficacy of therapeutic interventions on clinical symptoms in addition to declining motor abilities.<\/jats:p>","DOI":"10.1111\/gbb.12604","type":"journal-article","created":{"date-parts":[[2019,8,14]],"date-time":"2019-08-14T16:28:17Z","timestamp":1565800097000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Novel behavioural characteristics of the <i>superoxide dismutase 1 G93A<\/i> (<i>SOD1<\/i><sup><i>G93A<\/i><\/sup>) mouse model of amyotrophic lateral sclerosis include sex\u2010dependent phenotypes"],"prefix":"10.1111","volume":"19","author":[{"given":"Fabian","family":"Kreilaus","sequence":"first","affiliation":[{"name":"School of Medicine Western Sydney University  New South Wales 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