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

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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

DOI 10.1038/nn2073PMC PMC2895310

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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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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)
ORCID: Zhong, Zhihui
Deane, Rashid
Ali, Zarina
Sagare, Abhay
Cleveland, Don W
Venue: Nature Neuroscience
Publisher: Springer Science and Business Media LLC
Published: 2008-03-16
Volume: 11
Issue: 4
Pages: 420-422
ISSN: 1097-6256 (ISSN-L)
1546-1726
DOI: 10.1038/nn2073
PMC: PMC2895310
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: green
www.ncbi.nlm.nih.gov
Licence: www.springer.com/tdm (tdm)
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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ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration

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