Cited by 476Amyotrophic Lateral Sclerosis ResearchBarrier Structure and Function StudiesSpinal Cord Injury Research

The DOI matches its Crossref record; 15 of the 15 references it cites were found in a registry or an index.

ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration

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Zhong, Zhihui, Deane, Rashid, Ali, Zarina, Parisi, Margaret, Shapovalov, Yuriy, O'Banion, M Kerry, Stojanovic, Konstantin, Sagare, Abhay, Boillee, Severine, Cleveland, Don W, Zlokovic, Berislav V·2008·Nature Neuroscience

Abstract

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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Crossref · 10.1038/nn2073

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Metadata

Authors
Zhong, Z., Deane, R., Ali, Z., Parisi, M., Shapovalov, Y., O'Banion, M. K., Stojanovic, K., Sagare, A., Boillee, S., Cleveland, D. W., & Zlokovic, B. V.
Affiliations
  • University of Rochester Medicine (US)
  • Ludwig Cancer Research (BE)
Venue
Nature Neuroscience
Published
2008-03-16
Volume
11
Issue
4
Pages
420-422
ISSN
  • 1097-6256 (ISSN-L)
  • 1546-1726
OpenAlex
W1968758728
Cited by
476
References
15
Citations by year
2026: 12 · 2025: 18 · 2024: 29 · 2023: 26 · 2022: 20 · 2021: 33 · 2020: 27 · 2019: 26 · 2018: 22 · 2017: 18 · 2016: 18 · 2015: 25 · 2014: 32 · 2013: 28 · 2012: 31
FWCI
23.74
Type
journal article
Language
en
Open access
Locations
  • Nature Neuroscience (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • National Institute on Aging (R37AG023084)
  • National Heart, Lung, and Blood Institute (HL63290, R01HL063290)
  • National Institute of Neurological Disorders and Stroke (R37NS034467, R37 NS 27036, R37NS027036, R01NS034467, R37NS34467)
Topics
  • Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
  • Life Sciences › Neuroscience › Neurology › Barrier Structure and Function Studies
  • Health Sciences › Medicine › Clinical Neurology › Spinal Cord Injury Research
Keywords
amyotrophic lateral sclerosis, mutant SOD1, blood-spinal cord barrier, tight junction proteins, ZO-1, occludin, claudin-5, endothelial injury, microhemorrhages, microcirculation, hypoperfusion, neurovascular inflammation, motor neuron degeneration, disease initiation
MeSH terms
Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Blood-Brain Barrier, Capillaries, Endothelium, Vascular, Hemorrhage, Humans, Isoenzymes, Membrane Proteins, Mice, Mutant Strains, Motor Neurons, Nerve Degeneration, Phosphoproteins, Spinal Cord, Superoxide Dismutase, Disease Progression, Tight Junctions, Mice, Claudin-5, Occludin, Zonula Occludens-1 Protein

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