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Impaired Blood-Brain/Spinal Cord Barrier in ALS Patients
Brain Research
  • Svitlana N Garbuzova-Davis, University of South Florida
  • Diana Hernandez-Ontiveros, University of South Florida
  • Maria C. O. Rodrigues, University of South Florida
  • Edward M Haller, University of South Florida
  • Aric Frisna-Deyo, University of South Florida
  • Santhia Mirtyl, University of South Florida
  • Sebastian Sallot, University of South Florida
  • Samuel Saporta, University of South Florida
  • Cesario Borlongan, University of South Florida
  • Paul Sanberg, University of South Florida
Document Type
Publication Date
  • Amyotrophic lateral sclerosis,
  • Patients,
  • Blood–brain/spinal cord barrier,
  • Impairment
Digital Object Identifier (DOI)
Vascular pathology, including blood–brain/spinal cord barrier (BBB/BSCB) alterations, has recently been recognized as a key factor possibly aggravating motor neuron damage, identifying a neurovascular disease signature for ALS. However, BBB/BSCB competence in sporadic ALS (SALS) is still undetermined. In this study, BBB/BSCB integrity in postmortem gray and white matter of medulla and spinal cord tissue from SALS patients and controls was investigated. Major findings include (1) endothelial cell damage and pericyte degeneration, (2) severe intra- and extracellular edema, (3) reduced CD31 and CD105expressions in endothelium, (4) significant accumulation of perivascular collagen IV, and fibrin deposits (5) significantly increased microvascular density in lumbar spinal cord, (6) IgG microvascular leakage, (7) reduced tight junction and adhesion protein expressions. Microvascular barrier abnormalities determined in gray and white matter of the medulla, cervical, and lumbar spinal cord of SALS patients are novel findings. Pervasive barrier damage discovered in ALS may have implications for disease pathogenesis and progression, as well as for uncovering novel therapeutic targets. Highlights ► Capillary barrier integrity examined in postmortem tissue from ALS patients. ► Microvascular barrier abnormalities determined in CNS gray and white matter. ► Endothelial and pericyte cell degeneration compromised capillary barriers. ► Perivascular collagen accumulation and fibrin deposits determined. ► Pervasive barrier damage may have implications for disease pathogenesis.
Citation / Publisher Attribution

Brain Research, v. 1469, p. 114-128

Citation Information
Svitlana N Garbuzova-Davis, Diana Hernandez-Ontiveros, Maria C. O. Rodrigues, Edward M Haller, et al.. "Impaired Blood-Brain/Spinal Cord Barrier in ALS Patients" Brain Research Vol. 1469 (2012) p. 114 - 128
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