| First Author | Zhong Z | Year | 2008 |
| Journal | Nat Neurosci | Volume | 11 |
| Issue | 4 | Pages | 420-2 |
| PubMed ID | 18344992 | Mgi Jnum | J:136100 |
| Mgi Id | MGI:3795081 | Doi | 10.1038/nn2073 |
| Citation | Zhong Z, et al. (2008) ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci 11(4):420-2 |
| abstractText | 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. |
