ArticleProceedings of the National Academy of Sciences of the United States of America2001
Autoimmunity to beta IV spectrin in paraneoplastic lower motor neuron syndrome.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 49 citations in OpenAlex.
- Motor neuron disease can present as a paraneoplastic neurologic syndrome with various phenotypes.Brain communications · 2026Article
- The Broad Spectrum of Gallbladder Paraneoplastic Syndromes.Gastro hep advances · 2024Review
- Review
- Spectrins: molecular organizers and targets of neurological disorders.Nature reviews. Neuroscience · 2023Review
- Pathology of Initial Axon Segments in Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Related Disorders.International journal of molecular sciences · 2022Review
- βIV-Spectrin Autoantibodies in 2 Individuals With Neuropathy of Possible Paraneoplastic Origin: A Case Series.Neurology(R) neuroimmunology & neuroinflammation · 2022Article
- Dual ankyrinG and subpial autoantibodies in a man with well-controlled HIV infection with steroid-responsive meningoencephalitis: A case report.Frontiers in neurology · 2022Article
- Reorganization of Destabilized Nodes of Ranvier in βThe Journal of neuroscience : the official journal of the Society for Neuroscience · 2018Article
- Antibodies to TRIM46 are associated with paraneoplastic neurological syndromes.Annals of clinical and translational neurology · 2017Article
- Isoforms of Spectrin and Ankyrin Reflect the Functional Topography of the Mouse Kidney.PloS one · 2016Article
- Brca1 is expressed in human microglia and is dysregulated in human and animal model of ALS.Molecular neurodegeneration · 2015Article
- Node of Ranvier disruption as a cause of neurological diseases.ASN neuro · 2013Review
- Ankyrin facilitates intracellular trafficking of alpha1-Na+-K+-ATPase in polarized cells.American journal of physiology. Cell physiology · 2008Article
- BetaIVSigma1 spectrin stabilizes the nodes of Ranvier and axon initial segments.The Journal of cell biology · 2004Article
- Naturally occurring B-cell responses to breast cancer.Cancer immunology, immunotherapy : CII · 2003Review
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Paraneoplastic neurological disorders may result from autoimmunity directed against antigens shared by the affected neurons and the associated cancer cells. We have recently reported the case of a woman with breast cancer and paraneoplastic lower motor neuron syndrome whose serum contained autoantibodies directed against axon initial segments and nodes of Ranvier of myelinated axons, including the axons of motoneurons. Here, we show that major targets of the autoantibodies of this patient are betaIVSigma1 spectrin and betaIV spectrin 140, two isoforms of the novel betaIV spectrin gene, as well as a neuronal surface epitope yet to be identified. Partial improvement of the neurological symptoms following cancer removal was associated with a drastic reduction in the titer of the autoantibodies against betaIV spectrin and nodal antigens in general, consistent with the autoimmune pathogenesis of the paraneoplastic lower motor neuron syndrome. The identification of betaIV spectrin isoforms and surface nodal antigens as novel autoimmune targets in lower motor neuron syndrome provide new insights into the pathogenesis of this severe neurological disease.
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