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SM"]}]},{"name":"A Stafford Fox Medical Research Foundation Grant","award":["ARMC, France, contract R16009DD, to SM"],"award-info":[{"award-number":["ARMC, France, contract R16009DD, to SM"]}]},{"name":"A Stafford Fox Medical Research Foundation Grant","award":["FRC, France, contract AO-FRC 2009 #33536, to SM"],"award-info":[{"award-number":["FRC, France, contract AO-FRC 2009 #33536, to SM"]}]},{"name":"A Stafford Fox Medical Research Foundation Grant","award":["CT110725"],"award-info":[{"award-number":["CT110725"]}]},{"name":"The Florey Institute of Neuroscience and Mental Health acknowledges the strong support from the Victorian Government and in particular the funding from the Operational Infrastructure Support Grant","award":["ARSla, France, contract R11010DD, to PFP, contracts R17050DD, S.3200.ARSLA.1, to SM"],"award-info":[{"award-number":["ARSla, France, contract R11010DD, to PFP, contracts R17050DD, S.3200.ARSLA.1, to SM"]}]},{"name":"The Florey Institute of Neuroscience and Mental Health acknowledges the strong support from the Victorian Government and in particular the funding from the Operational Infrastructure Support Grant","award":["AFM, France, #19466, to SM"],"award-info":[{"award-number":["AFM, France, #19466, to SM"]}]},{"name":"The Florey Institute of Neuroscience and Mental Health acknowledges the strong support from the Victorian Government and in particular the funding from the Operational Infrastructure Support Grant","award":["ARMC, France, contract R16009DD, to SM"],"award-info":[{"award-number":["ARMC, France, contract R16009DD, to SM"]}]},{"name":"The Florey Institute of Neuroscience and Mental Health acknowledges the strong support from the Victorian Government and in particular the funding from the Operational Infrastructure Support Grant","award":["FRC, France, contract AO-FRC 2009 #33536, to SM"],"award-info":[{"award-number":["FRC, France, contract AO-FRC 2009 #33536, to SM"]}]},{"name":"The Florey Institute of Neuroscience and Mental Health acknowledges the strong support from the Victorian Government and in particular the funding from the Operational Infrastructure Support Grant","award":["CT110725"],"award-info":[{"award-number":["CT110725"]}]},{"name":"PhD Fellowship from AFM (#18145) and benefited from a student exchange program between ICM (France) and the Florey Institute of Neuroscience and Mental Health (Australia)","award":["ARSla, France, contract R11010DD, to PFP, contracts R17050DD, S.3200.ARSLA.1, to SM"],"award-info":[{"award-number":["ARSla, France, contract R11010DD, to PFP, contracts R17050DD, S.3200.ARSLA.1, to SM"]}]},{"name":"PhD Fellowship from AFM (#18145) and benefited from a student exchange program between ICM (France) and the Florey Institute of Neuroscience and Mental Health (Australia)","award":["AFM, France, #19466, to SM"],"award-info":[{"award-number":["AFM, France, #19466, to SM"]}]},{"name":"PhD Fellowship from AFM (#18145) and benefited from a student exchange program between ICM (France) and the Florey Institute of Neuroscience and Mental Health (Australia)","award":["ARMC, France, contract R16009DD, to SM"],"award-info":[{"award-number":["ARMC, France, contract R16009DD, to SM"]}]},{"name":"PhD Fellowship from AFM (#18145) and benefited from a student exchange program between ICM (France) and the Florey Institute of Neuroscience and Mental Health (Australia)","award":["FRC, France, contract AO-FRC 2009 #33536, to SM"],"award-info":[{"award-number":["FRC, France, contract AO-FRC 2009 #33536, to SM"]}]},{"name":"PhD Fellowship from AFM (#18145) and benefited from a student exchange program between ICM (France) and the Florey Institute of Neuroscience and Mental Health (Australia)","award":["CT110725"],"award-info":[{"award-number":["CT110725"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Mutations in profilin 1 (PFN1) have been identified in rare familial cases of Amyotrophic Lateral Sclerosis (ALS). PFN1 is involved in multiple pathways that could intervene in ALS pathology. However, the specific pathogenic role of PFN1 mutations in ALS is still not fully understood. We hypothesized that PFN1 could play a role in regulating autophagy pathways and that PFN1 mutations could disrupt this function. We used patient cells (lymphoblasts) or tissue (post-mortem) carrying PFN1 mutations (M114T and E117G), and designed experimental models expressing wild-type or mutant PFN1 (cell lines and novel PFN1 mice established by lentiviral transgenesis) to study the effects of PFN1 mutations on autophagic pathway markers. We observed no accumulation of PFN1 in the spinal cord of one E117G mutation carrier. Moreover, in patient lymphoblasts and transfected cell lines, the M114T mutant PFN1 protein was unstable and deregulated the RAB9-mediated alternative autophagy pathway involved in the clearance of damaged mitochondria. In vivo, motor neurons expressing M114T mutant PFN1 showed mitochondrial abnormalities. Our results demonstrate that the M114T PFN1 mutation is more deleterious than the E117G variant in patient cells and experimental models and suggest a role for the RAB9-dependent autophagic pathway in ALS.<\/jats:p>","DOI":"10.3390\/ijms23105694","type":"journal-article","created":{"date-parts":[[2022,5,19]],"date-time":"2022-05-19T12:25:57Z","timestamp":1652963157000},"page":"5694","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["The Amyotrophic Lateral Sclerosis M114T PFN1 Mutation Deregulates Alternative Autophagy Pathways and Mitochondrial Homeostasis"],"prefix":"10.3390","volume":"23","author":[{"given":"Elisa","family":"Teyssou","sequence":"first","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Chartier","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4644-7679","authenticated-orcid":false,"given":"Delphine","family":"Roussel","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7695-6290","authenticated-orcid":false,"given":"Nirma D.","family":"Perera","sequence":"additional","affiliation":[{"name":"The Florey Institute of Neuroscience and Mental Health, University of Melbourne, Parkville, VIC 3052, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8080-839X","authenticated-orcid":false,"given":"Ivan","family":"Nemazanyy","sequence":"additional","affiliation":[{"name":"Platform for Metabolic Analyses, Structure F\u00e9d\u00e9rative de Recherche Necker, INSERM US24\/CNRS UAR 3633, F-75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5484-2359","authenticated-orcid":false,"given":"Dominique","family":"Langui","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e9lanie","family":"Albert","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thierry","family":"Larmonier","sequence":"additional","affiliation":[{"name":"Banque d\u2019ADN et de Cellules du G\u00e9n\u00e9thon, F-91000 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Safaa","family":"Saker","sequence":"additional","affiliation":[{"name":"Banque d\u2019ADN et de Cellules du G\u00e9n\u00e9thon, F-91000 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Salachas","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"},{"name":"Centre de R\u00e9f\u00e9rence SLA Ile de France, D\u00e9partement de Neurologie, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re, APHP, DMU Neuroscience, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre-Fran\u00e7ois","family":"Pradat","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence SLA Ile de France, D\u00e9partement de Neurologie, H\u00f4pital de la Piti\u00e9-Salp\u00eatri\u00e8re, APHP, DMU Neuroscience, F-75013 Paris, France"},{"name":"Laboratoire d\u2019Imagerie Biom\u00e9dicale, Sorbonne Universit\u00e9, INSERM UMRS1146, CNRS UMR7371, F-75013 Paris, France"},{"name":"Northern Ireland Centre for Stratified Medicine, Biomedical Sciences Research Institute Ulster University, C-TRIC, Altnagelvin Hospital, Derry-Londonderry BT47 6SB, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Meininger","sequence":"additional","affiliation":[{"name":"H\u00f4pital des Peupliers, Ramsay G\u00e9n\u00e9rale de Sant\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Ravassard","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francine","family":"C\u00f4t\u00e9","sequence":"additional","affiliation":[{"name":"Institut Cochin, INSERM U1016, CNRS UMR8104, Universit\u00e9 de Paris, F-75014 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian S.","family":"Lobsiger","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9verine","family":"Boill\u00e9e","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6580-7655","authenticated-orcid":false,"given":"Bradley J.","family":"Turner","sequence":"additional","affiliation":[{"name":"The Florey Institute of Neuroscience and Mental Health, University of Melbourne, Parkville, VIC 3052, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Seilhean","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"},{"name":"APHP, D\u00e9partement de Neuropathologie, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, DMU Neuroscience, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0745-6735","authenticated-orcid":false,"given":"St\u00e9phanie","family":"Millecamps","sequence":"additional","affiliation":[{"name":"Institut du Cerveau\u2014Paris Brain Institute\u2014ICM, Inserm, CNRS, APHP, H\u00f4pital de la Piti\u00e9 Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, F-75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1016\/j.molmed.2016.07.005","article-title":"Common Molecular Pathways in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia","volume":"22","author":"Weishaupt","year":"2016","journal-title":"Trends Mol. 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