ArticleInternational journal of molecular sciences2020
Human Spinal Motor Neurons Are Particularly Vulnerable to Cerebrospinal Fluid of Amyotrophic Lateral Sclerosis Patients.
Article in International journal of molecular sciences, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
7 citing papers in PubMed, 8 citations in OpenAlex.
- Corrosion Products from Metallic Implants Induce ROS and Cell Death in Human MotoneuronsJournal of functional biomaterials · 2023Article
- Deciphering the impact of cerebrospinal fluid on stem cell fate as a new mechanism to enhance clinical therapy development.Frontiers in neuroscience · 2023Review
- Storage of Mutant Human SOD1 in Non-Neural Cells from the Type-1 Amyotrophic Lateral Sclerosis ratBiomedicines · 2021Article
- hiPSC-Derived Cells as Models for Drug Discovery.International journal of molecular sciences · 2021Article
- 40 Years of CSF Toxicity Studies in ALS: What Have We Learnt About ALS Pathophysiology?Frontiers in molecular neuroscience · 2021Review
- Defining novel functions for cerebrospinal fluid in ALS pathophysiology.Acta neuropathologica communications · 2020Review
- Cerebrospinal fluid cytotoxicity in amyotrophic lateral sclerosis: a systematic review ofBrain communications · 2020Review
Corrections and comments
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Authors and funding
5 authors at 4 institutions in 1 country.
Funding
Abstract
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-a typical finding in lower organism models and human postmortem tissue-was induced solely by addition of ALS-CSF, but not control-CSF. No other neurodegenerative hallmarks-including pathological protein aggregation-were 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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.