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ALS is characterized by the selective and progressive loss of motoneurons in the spinal cord, brainstem and cerebral cortex. Clinical manifestations typically occur in midlife and start with focal muscle weakness, followed by the rapid and progressive wasting of muscles and subsequent paralysis. As with other neurodegenerative diseases, the condition typically begins at an initial point and then spreads along neuroanatomical tracts. This feature of disease progression suggests the spreading of prion-like proteins called prionoids in the affected tissues, which is similar to the spread of prion observed in Creutzfeldt-Jakob disease. Intensive research over the last decade has proposed the ALS-causing gene products Cu\/Zn superoxide dismutase 1, TAR DNA-binding protein of 43\u2005kDa, and fused in sarcoma as very plausible prionoids contributing to the spread of the pathology. In this review, we will discuss the molecular and cellular mechanisms leading to the propagation of these prionoids in ALS.<\/jats:p>","DOI":"10.1093\/braincomms\/fcac145","type":"journal-article","created":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T12:40:45Z","timestamp":1654778445000},"source":"Crossref","is-referenced-by-count":24,"title":["Prionoids in amyotrophic lateral sclerosis"],"prefix":"10.1093","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9104-8688","authenticated-orcid":false,"given":"Philippe","family":"Gosset","sequence":"first","affiliation":[{"name":"INM, Univ Montpellier, INSERM, CNRS , Montpellier 34095, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Camu","sequence":"additional","affiliation":[{"name":"INM, Univ Montpellier, INSERM, CNRS , Montpellier 34095, France"},{"name":"ALS Center, Univ Montpellier, CHU Montpellier , Montpellier, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0561-6851","authenticated-orcid":false,"given":"Cedric","family":"Raoul","sequence":"additional","affiliation":[{"name":"INM, Univ Montpellier, INSERM, CNRS , Montpellier 34095, France"},{"name":"Laboratory of Neurobiology, Kazan Federal University , Kazan, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Mezghrani","sequence":"additional","affiliation":[{"name":"INM, Univ Montpellier, INSERM, CNRS , Montpellier 34095, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,6,9]]},"reference":[{"issue":"10","key":"2022062919005963600_fcac145-B1","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1038\/s41593-018-0238-6","article-title":"Propagation and spread of pathogenic protein assemblies in neurodegenerative 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