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| Content Provider | Springer Nature : BioMed Central |
|---|---|
| Author | Aragón-González, Ana Shaw, Allan C Kok, Jannigje R Roussel, Florence S Santos Souza, Cleide dos Granger, Sarah M Vetter, Tatyana de Diego, Yolanda Meyer, Kathrin C Beal, Selina N Shaw, Pamela J Ferraiuolo, Laura |
| Abstract | The blood-brain barrier (BBB) serves as a highly intricate and dynamic interface connecting the brain and the bloodstream, playing a vital role in maintaining brain homeostasis. BBB dysfunction has been associated with multiple neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS); however, the role of the BBB in neurodegeneration is understudied. We developed an ALS patient-derived model of the BBB by using cells derived from 5 patient donors carrying C9ORF72 mutations. Brain microvascular endothelial-like cells (BMEC-like cells) derived from C9ORF72-ALS patients showed altered gene expression, compromised barrier integrity, and increased P-glycoprotein transporter activity. In addition, mitochondrial metabolic tests demonstrated that C9ORF72-ALS BMECs display a significant decrease in basal glycolysis accompanied by increased basal and ATP-linked respiration. Moreover, our study reveals that C9-ALS derived astrocytes can further affect BMECs function and affect the expression of the glucose transporter Glut-1. Finally, C9ORF72 patient-derived BMECs form leaky barriers through a cell-autonomous mechanism and have neurotoxic properties towards motor neurons. Graphical Abstract |
| Related Links | https://fluidsbarrierscns.biomedcentral.com/counter/pdf/10.1186/s12987-024-00528-6.pdf |
| Ending Page | 14 |
| Page Count | 14 |
| Starting Page | 1 |
| File Format | HTM / HTML |
| ISSN | 20458118 |
| DOI | 10.1186/s12987-024-00528-6 |
| Journal | Fluids and Barriers of the CNS |
| Issue Number | 1 |
| Volume Number | 21 |
| Language | English |
| Publisher | BioMed Central |
| Publisher Date | 2024-04-11 |
| Access Restriction | Open |
| Subject Keyword | Neurosciences Hematology Neurobiology In vitro modelling Stem cells Blood brain barrier Amyotrophic lateral sclerosis C9ORF72 Neurodegeneration |
| Content Type | Text |
| Resource Type | Article |
| Subject | Neurology Developmental Neuroscience Medicine Cellular and Molecular Neuroscience |
| Journal Impact Factor | 5.9/2023 |
| 5-Year Journal Impact Factor | 7.5/2023 |
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