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Autoimmunity to βIV Spectrin in Paraneoplastic Lower Motor Neuron Syndrome
Stanny Berghs, Franco Ferracci, Elena Maksimova, Shannon Gleason, Nancy Leszczynski, Margaret Butler, Pietro De Camilli and Michele Solimena
Proceedings of the National Academy of Sciences of the United States of America
Vol. 98, No. 12 (Jun. 5, 2001), pp. 6945-6950
Published by: National Academy of Sciences
Stable URL: http://www.jstor.org/stable/3055917
Page Count: 6
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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 βIVΣ1 spectrin and βIV spectrin 140, two isoforms of the novel βIV 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 βIV spectrin and nodal antigens in general, consistent with the autoimmune pathogenesis of the paraneoplastic lower motor neuron syndrome. The identification of βIV 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.
Proceedings of the National Academy of Sciences of the United States of America © 2001 National Academy of Sciences