{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T02:50:46Z","timestamp":1760237446838,"version":"build-2065373602"},"reference-count":50,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2020,5,18]],"date-time":"2020-05-18T00:00:00Z","timestamp":1589760000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Schilling Stiftung im Stifterverband","award":["none"],"award-info":[{"award-number":["none"]}]},{"name":"Deutsche Gesellschaft f\u00fcr Muskelerkrankungen","award":["He2\/2"],"award-info":[{"award-number":["He2\/2"]}]},{"DOI":"10.13039\/501100008483","name":"NOMIS Foundation","doi-asserted-by":"publisher","award":["none"],"award-info":[{"award-number":["none"]}],"id":[{"id":"10.13039\/501100008483","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Helmholtz Virtual Institute \u201cRNA dysmetabolism in ALS and FTD","award":["VH-VI-510"],"award-info":[{"award-number":["VH-VI-510"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Amyotrophic lateral sclerosis (ALS) is the most common and devastating motor neuron (MN) disease. Its pathophysiological cascade is still enigmatic. More than 90% of ALS patients suffer from sporadic ALS, which makes it specifically demanding to generate appropriate model systems. One interesting aspect considering the seeding, spreading and further disease development of ALS is the cerebrospinal fluid (CSF). We therefore asked whether CSF from sporadic ALS patients is capable of causing disease typical changes in human patient-derived spinal MN cultures and thus could represent a novel model system for sporadic ALS. By using induced pluripotent stem cell (iPSC)-derived MNs from healthy controls and monogenetic forms of ALS we could demonstrate a harmful effect of ALS-CSF on healthy donor-derived human MNs. Golgi fragmentation\u2014a typical finding in lower organism models and human postmortem tissue\u2014was induced solely by addition of ALS-CSF, but not control-CSF. No other neurodegenerative hallmarks\u2014including pathological protein aggregation\u2014were found, underpinning Golgi fragmentation as early event in the neurodegenerative cascade. Of note, these changes occurred predominantly in MNs, the cell type primarily affected in ALS. We thus present a novel way to model early features of sporadic ALS.<\/jats:p>","DOI":"10.3390\/ijms21103564","type":"journal-article","created":{"date-parts":[[2020,5,18]],"date-time":"2020-05-18T11:34:14Z","timestamp":1589801654000},"page":"3564","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Human Spinal Motor Neurons Are Particularly Vulnerable to Cerebrospinal Fluid of Amyotrophic Lateral Sclerosis Patients"],"prefix":"10.3390","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2449-5530","authenticated-orcid":false,"given":"Stefan","family":"Br\u00e4uer","sequence":"first","affiliation":[{"name":"Department of Neurology, Technische Universit\u00e4t Dresden, 01307 Dresden, Germany"},{"name":"Department of Neurology, St\u00e4dtisches Klinikum Dresden, 01129 Dresden, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ren\u00e9","family":"G\u00fcnther","sequence":"additional","affiliation":[{"name":"Department of Neurology, Technische Universit\u00e4t Dresden, 01307 Dresden, Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE), 01307 Dresden, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jared","family":"Sterneckert","sequence":"additional","affiliation":[{"name":"Center for Regenerative Therapies Dresden (CRTD), Technische Universit\u00e4t Dresden, 01307 Dresden, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3155-5300","authenticated-orcid":false,"given":"Hannes","family":"Gla\u00df","sequence":"additional","affiliation":[{"name":"Translational Neurodegeneration Section \u201cAlbrecht-Kossel\u201d, Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7364-7791","authenticated-orcid":false,"given":"Andreas","family":"Hermann","sequence":"additional","affiliation":[{"name":"Department of Neurology, Technische Universit\u00e4t Dresden, 01307 Dresden, Germany"},{"name":"Translational Neurodegeneration Section \u201cAlbrecht-Kossel\u201d, Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) Rostock, 18147 Rostock, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,5,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1007\/s00702-018-1851-y","article-title":"Prion-like properties of disease-relevant proteins in amyotrophic lateral sclerosis","volume":"125","author":"Brauer","year":"2018","journal-title":"J. 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