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Cited by 2,288Multiple Sclerosis Research StudiesIon Transport and Channel RegulationPeripheral Neuropathies and Disorders

IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel

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Lennon, Vanda A., Kryzer, Thomas J., Pittock, Sean J., Verkman, A.S., Hinson, Shannon R.·2005·The Journal of Experimental Medicine
DOI 10.1084/jem.20050304PMC PMC2212860
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Abstract

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. It is considered a severe variant of multiple sclerosis (MS), and frequently is misdiagnosed as MS, but prognosis and optimal treatments differ. A serum immunoglobulin G autoantibody (NMO-IgG) serves as a specific marker for NMO. Here we show that NMO-IgG binds selectively to the aquaporin-4 water channel, a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier. NMO may represent the first example of a novel class of autoimmune channelopathy.

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  • Recent data suggest that some AQP4-seronegative patients with NMOSD features have antibodies reactive with MOG.

    “Recent data suggest that some AQP4-seronegative patients with NMOSD features have antibodies reactive with MOG. 47-51”

    From Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria (2018) · cites this paper as [51]

    The Abstract reports that NMO-IgG binds AQP4 and was detected in 73% of patients with NMO. Neither it nor the Results and Discussion passages address whether AQP4-seronegative patients have antibodies reactive with MOG; searches for MOG and seronegative patients found no such finding in our copy. (LLM, conf=0.76)

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References 32 · Cited by 2,288

Research works printed but not linked: 27

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Authors
Lennon, V. A., Kryzer, T. J., Pittock, S. J., Verkman, A. S., & Hinson, S. R.
Affiliations
  • Mayo Clinic (US)
  • University of California, San Francisco (US)
ORCID
Venue
The Journal of Experimental Medicine
Publisher
Rockefeller University Press
Published
2005-08-08
Volume
202
Issue
4
Pages
473-477
ISSN
  • 0022-1007 (ISSN-L)
  • 1540-9538
DOI
10.1084/jem.20050304
PMC
PMC2212860
OpenAlex
W2144469764
Cited by
2,288
References
32
Citations by year
2026: 88 · 2025: 90 · 2024: 88 · 2023: 84 · 2022: 97 · 2021: 119 · 2020: 115 · 2019: 107 · 2018: 93 · 2017: 110 · 2016: 113 · 2015: 128 · 2014: 129 · 2013: 137 · 2012: 147
FWCI
58.97
Type
journal article
Language
en
Open access
Locations
  • The Journal of Experimental Medicine (publishedVersion)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by-nc-sa)
  • PubMed Central (publishedVersion, cc-by-nc-sa)
  • PubMed (publishedVersion)
  • eScholarship (California Digital Library) (submittedVersion)
  • eScholarship (California Digital Library) (submittedVersion, cc-by-nc-sa)
Topics
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion Transport and Channel Regulation
  • Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
Keywords
neuromyelitis optica, multiple sclerosis, aquaporin-4 antibodies, aquaporin-4, autoantibodies, dystroglycan complex, astrocytes, blood-brain barrier, demyelination, optic nerve, spinal cord
MeSH terms
Animals, Astrocytes, Autoantibodies, Blood-Brain Barrier, Humans, Immunoglobulin G, Mice, Mutant Strains, Neuromyelitis Optica, Optic Nerve, Protein Binding, Spinal Cord, Biomarkers, Aquaporins, Multiprotein Complexes, Dystroglycans, Mice, Rats, Aquaporin 4

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