A new paraneoplastic encephalomyelitis autoantibody reactive with the axon initial segment |
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Authors: | Setareh Shams’ili Bertie de Leeuw Esther Hulsenboom Dick Jaarsma Peter Sillevis Smitt |
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Institution: | 1. Department of Neurology, Erasmus University Medical Center, The Netherlands;2. Department of Neuroscience, Erasmus University Medical Center, Rotterdam, The Netherlands |
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Abstract: | Serum from a patient with paraneoplastic encephalomyelitis (PEM) and small cell lung cancer (SCLC) showed high titer immunohistochemical staining of the axon initial segment (AIS) on rat and human brain sections. EM studies showed that the antigen was localized in close proximity of the microtubules in the AIS. Double labeling experiments and absence of staining at the nodes of Ranvier excluded the previously identified βIV spectrin as autoantigen. Screening a rat hippocampal cDNA library resulted in the isolation of ubiquitin-conjugating enzyme E2E1 (UBE2E1). However, blocking and elution experiments excluded UBE2E1 as the AIS autoantigen. |
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Keywords: | Paraneoplastic encephalomyelitis Axon initial segment UBE2E1 UbcH6 βIV spectrin |
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