Impaired blood–brain and blood–spinal cord barriers in mutant SOD1-linked ALS rat
Open accessAbstract
Blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB) impairment is an additional accident occurring during the amyotrophic lateral sclerosis (ALS) progression. In this work, we aimed to decipher if BBB/BSCB leakage appeared before critical detrimental events and could serve as a marker preceding clinical symptoms. Three different BBB leakage markers: Evans blue, IgG and hemosiderin, were used to look at the SOD1-linked ALS rat model at presymptomatic and symptomatic stages. Although IgG and hemosiderin could be detected at presymptomatic stage, Evans blue extravasation which fits best with BBB/BSCB impairment could only be seen at symptomatic stages. BBB/BSCB impairment was further substantiate by showing at symptomatic stages decreased mRNA expression of ZO-1 and occludin as well as agrin, a basal membrane constituent. Electron microscopic data substantiate a toxic environment around endothelial cell and peri-vascular swollen astrocyte end-feet showing oedema-linked BBB opening.
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Early BBB breakdown was also shown in SOD1 G93A rats at a presymptomatic stage and has been confirmed using different contrast-enhanced MRI techniques.
“Early BBB breakdown was also shown in SOD1 G93A rats at a presymptomatic stage (431) and has been confirmed using different contrast-enhanced MRI techniques (20, 51).”
From Blood-Brain Barrier: From Physiology to Disease and Back (2019) · cites this paper as [431]
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- Authors
- Nicaise, C., Mitrecic, D., Demetter, P., De Decker, R., Authelet, M., Boom, A., & Pochet, R.
- Affiliations
- Université Libre de Bruxelles (BE)
- Erasmus Hospital (BE)
- Venue
- Brain Research
- Publisher
- Elsevier BV
- Published
- 2009-11
- Volume
- 1301
- Pages
- 152-162
- ISSN
- 0006-8993 (ISSN-L)
- OpenAlex
- W2149180455
- Cited by
- 168
- References
- 39
- Citations by year
- 2026: 3 · 2025: 8 · 2024: 14 · 2023: 7 · 2022: 5 · 2021: 18 · 2020: 7 · 2019: 9 · 2018: 11 · 2017: 8 · 2016: 4 · 2015: 11 · 2014: 14 · 2013: 10 · 2012: 19
- FWCI
- 8.31
- Type
- journal article
- Language
- en
- Open access
- green
- hdl.handle.net
- Licence
- Locations
- Brain Research (publishedVersion)
- PubMed (publishedVersion)
- Repository of the University of Namur (publishedVersion)
- Dépôt institutionnel de l'Université libre de Bruxelles (Université Libre de Bruxelles) (submittedVersion, other-oa)
- Topics
- Life Sciences › Neuroscience › Neurology › Barrier Structure and Function Studies
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
- Keywords
- amyotrophic lateral sclerosis, blood-brain barrier, blood-spinal cord barrier, BBB leakage, Evans blue extravasation, hemosiderin, ZO-1, occludin, agrin, endothelial tight junctions, astrocyte endfeet
- MeSH terms
- Amyotrophic Lateral Sclerosis, Animals, Astrocytes, Blood-Brain Barrier, Brain Stem, Disease Models, Animal, Enzyme-Linked Immunosorbent Assay, Evans Blue, Hemosiderin, Immunoglobulins, Immunohistochemistry, Membrane Proteins, Microscopy, Electron, Phosphoproteins, RNA, Messenger, Spinal Cord, Staining and Labeling, Superoxide Dismutase, Biomarkers, Agrin, Reverse Transcriptase Polymerase Chain Reaction, Endothelial Cells, Cell Shape, Rats, Rats, Transgenic, Occludin, Zonula Occludens-1 Protein
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