Impaired blood-brain/spinal cord barrier in ALS patients
Contribuinte(s) |
UNIVERSIDADE DE SÃO PAULO |
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Data(s) |
31/10/2013
31/10/2013
02/08/2013
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Resumo |
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 CD105 expressions 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. (C) 2012 Elsevier B.V. All rights reserved. Muscular Dystrophy Association [92452] Muscular Dystrophy Association |
Identificador |
BRAIN RESEARCH, AMSTERDAM, v. 1469, n. 1, supl. 1, Part 6, pp. 114-128, AUG 21, 2012 0006-8993 http://www.producao.usp.br/handle/BDPI/37113 10.1016/j.brainres.2012.05.056 |
Idioma(s) |
eng |
Publicador |
ELSEVIER SCIENCE BV AMSTERDAM |
Relação |
BRAIN RESEARCH |
Direitos |
closedAccess Copyright ELSEVIER SCIENCE BV |
Palavras-Chave | #AMYOTROPHIC LATERAL SCLEROSIS #PATIENTS #BLOOD-BRAIN/SPINAL CORD BARRIER #IMPAIRMENT #AMYOTROPHIC-LATERAL-SCLEROSIS #MOTOR-NEURON DEGENERATION #MICE MODELING ALS #MATRIX METALLOPROTEINASES #BRAIN-BARRIER #SPINAL-CORD #TISSUE INHIBITORS #CEREBROSPINAL-FLUID #CEREBRAL-ISCHEMIA #DISEASE #NEUROSCIENCES |
Tipo |
article original article publishedVersion |