Journal
NATURE NEUROSCIENCE
Volume 11, Issue 4, Pages 420-422Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nn2073
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Funding
- NHLBI NIH HHS [R01 HL063290, R01 HL063290-01, HL63290] Funding Source: Medline
- NIA NIH HHS [R37 AG023084-01A1, R37 AG023084] Funding Source: Medline
- NINDS NIH HHS [R37 NS027036, R37 NS034467-10, R37 NS034467, R37 NS27036, R37 NS34467, R01 NS034467] Funding Source: Medline
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We report here that amyotrophic lateral sclerosis-linked superoxide dismutase 1 (SOD1) mutants with different biochemical characteristics disrupted the blood-spinal cord barrier in mice by reducing the levels of the tight junction proteins ZO-1, occludin and claudin-5 between endothelial cells. This resulted in microhemorrhages with release of neurotoxic hemoglobin-derived products, reductions in microcirculation and hypoperfusion. SOD1 mutant-mediated endothelial damage accumulated before motor neuron degeneration and the neurovascular inflammatory response occurred, indicating that it was a central contributor to disease initiation.
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