Impaired Blood-Brain/Spinal Cord Barrier in ALS Patients
Document Type
Article
Publication Date
8-21-2012
Keywords
Amyotrophic lateral sclerosis, Patients, Blood–brain/spinal cord barrier, Impairment
Digital Object Identifier (DOI)
https://doi.org/10.1016/j.brainres.2012.05.056
Abstract
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.
Was this content written or created while at USF?
Yes
Citation / Publisher Attribution
Brain Research, v. 1469, p. 114-128
Scholar Commons Citation
Garbuzova-Davis, Svitlana N.; Hernandez-Ontiveros, Diana; Rodrigues, Maria C. O.; Haller, Edward M.; Frisna-Deyo, Aric; Mirtyl, Santhia; Sallot, Sebastian; Saporta, Samuel; Borlongan, Cesario; and Sanberg, Paul, "Impaired Blood-Brain/Spinal Cord Barrier in ALS Patients" (2012). Integrative Biology Faculty and Staff Publications. 284.
https://digitalcommons.usf.edu/bin_facpub/284
