Impaired blood-brain and blood-spinal cord barriers in mutant SOD1-linked ALS rat

Charles Nicaise, Dinko Mitrecic, Pieter Demetter, Robert De Decker, Michèle Authelet, Alain Boom, Roland Pochet

Research output: Contribution to journalArticlepeer-review


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.
Original languageEnglish
Pages (from-to)152-62
Number of pages11
JournalBrain research
Publication statusPublished - 8 Dec 2009


  • Agrin
  • Amyotrophic Lateral Sclerosis
  • Animals
  • Astrocytes
  • Biological Markers
  • Blood-Brain Barrier
  • Brain Stem
  • Cell Shape
  • Disease Models, Animal
  • Endothelial Cells
  • Enzyme-Linked Immunosorbent Assay
  • Evans Blue
  • Hemosiderin
  • Immunoglobulins
  • Immunohistochemistry
  • Membrane Proteins
  • Microscopy, Electron
  • Occludin
  • Phosphoproteins
  • RNA, Messenger
  • Rats
  • Rats, Transgenic
  • Reverse Transcriptase Polymerase Chain Reaction
  • Spinal Cord
  • Staining and Labeling
  • Superoxide Dismutase
  • Zonula Occludens-1 Protein


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